Uterine sarcoma: Professional resources from the National Cancer Institute

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Uterine sarcoma treatment

Uterine Sarcoma Treatment (PDQ®)–Health Professional Version

General Information About Uterine Sarcoma

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Uterine sarcomas comprise less than 1% of gynecologic malignancies and 2% to 5% of all uterine malignancies.[1] The following tumors arise primarily from three distinct tissues:

  1. Carcinosarcomas arising in the endometrium, in other organs of mullerian origin, and accounting for 40% to 50% of all uterine sarcomas.
  2. Leiomyosarcomas arising from myometrial muscle, with a peak incidence occurring at age 50, and accounting for 30% of all uterine sarcomas.
  3. Sarcomas arising in the endometrial stroma, with a peak incidence occurring before menopause for the low-grade tumors and after menopause for the high-grade tumors, and accounting for 15% of all uterine sarcomas.

The three distinct entities are often grouped under uterine sarcomas; however, each type of tumor is currently being studied in separate clinical trials.

Carcinosarcomas (the preferred designation by the World Health Organization [WHO]) are also referred to as mixed mesodermal sarcomas or mullerian tumors. Controversy exists about the following issues:

  • Whether they are true sarcomas.
  • Whether the sarcomatous elements are actually derived from a common epithelial-cell precursor that also gives rise to the usually more abundant adenocarcinomatous elements.

The stromal components of the carcinosarcomas are further characterized by whether they contain homologous elements, such as malignant mesenchymal tissue considered possibly native to the uterus, or heterologous elements, such as striated muscle, cartilage, or bone, which are foreign to the uterus. Carcinosarcomas parallel endometrial cancer in its postmenopausal predominance and in other of its epidemiologic features; increasingly, the treatment of carcinosarcomas is becoming similar to combined modality approaches for endometrial adenocarcinomas.

Other rare forms of uterine sarcomas also fall under the WHO classification of mesenchymal and mixed tumors of the uterus. These include:[2,3]

  • Mixed endometrial stromal and smooth muscle tumors.
  • Adenosarcomas, in which the epithelial elements appear benign within a malignant mesenchymal background.
  • Embryonal botryoides or rhabdomyosarcomas, which are found almost exclusively in infants.
  • PEComa—a perivascular epithelial-cell tumor that may behave in a malignant fashion, which is the latest to be added.

(Refer to the PDQ summary on Childhood Rhabdomyosarcoma for more information.)

Risk Factors

The only documented etiologic factor in 10% to 25% of these malignancies is prior pelvic radiation therapy, which is often administered for benign uterine bleeding that began 5 to 25 years earlier. An increased incidence of uterine sarcoma has been associated with tamoxifen in the treatment of breast cancer. Subsequently, increases have also been noted when tamoxifen was given to prevent breast cancer in women at increased risk—a possible result of the estrogenic effect of tamoxifen on the uterus. Because of this increase, patients on tamoxifen should have follow-up pelvic examinations and should undergo endometrial biopsy if there is any abnormal uterine bleeding.[4-6]

Prognosis

The prognosis for women with uterine sarcoma is primarily dependent on the extent of disease at the time of diagnosis.[7] For women with carcinosarcomas, significant predictors of metastatic disease at initial surgery include:[7]

  • Isthmic or cervical location.
  • Lymphatic vascular space invasion.
  • Serous and clear cell histology.
  • Grade 2 or 3 carcinoma.

The above factors in addition to the following ones correlate with a progression-free interval:[7]

  • Adnexal spread.
  • Lymph node metastases.
  • Tumor size.
  • Peritoneal cytologic findings.
  • Depth of myometrial invasion.

Factors that bear no relationship to the presence or absence of metastases at surgical exploration are:

  • The presence or absence of stromal heterologous elements.
  • The types of such elements.
  • The grade of the stromal components.
  • The mitotic activity of the stromal components.

In one study, women with a well-differentiated sarcomatous component or carcinosarcomas had significantly longer progression-free intervals than those with moderately to poorly differentiated sarcomas for the homologous and heterologous types. The recurrence rate was 44% for homologous tumors and 63% for heterologous tumors. The type of heterologous sarcoma had no effect on the progression-free interval.

For women with leiomyosarcomas, some investigators consider tumor size to be the most important prognostic factor; women with tumors greater than 5.0 cm in maximum diameter have a poor prognosis.[8] However, in a Gynecologic Oncology Group study, the mitotic index was the only factor significantly related to progression-free interval.[7] Leiomyosarcomas matched for other known prognostic factors may be more aggressive than their carcinosarcoma counterparts.[9] The 5-year survival rate for women with stage I disease, which is confined to the corpus, is approximately 50% versus 0% to 20% for the remaining stages.

Surgery alone can be curative if the malignancy is contained within the uterus. The value of pelvic radiation therapy is not established. Current studies consist primarily of phase II chemotherapy trials for patients with advanced disease. Adjuvant chemotherapy following complete resection for patients with stage I or II disease was not established to be effective in a randomized trial.[10] Yet, other nonrandomized trials have reported improved survival following adjuvant chemotherapy with or without radiation therapy.[11-13]

Related Summaries

Other PDQ summaries containing information related to uterine sarcoma include the following:

References
  1. Forney JP, Buschbaum HJ: Classifying, staging, and treating uterine sarcomas. Contemp Ob Gyn 18(3):47, 50, 55-56, 61-62, 64, 69, 1981.
  2. Gershenson D, McGuire W, Gore Martin, et al.: Gynecologic Cancer: Controversies in Management. 3rd ed. New York, NY: Churchill Livingstone, 2004.
  3. Tavassoéli F, Devilee P, et al.: Pathology and Genetics of Tumours of the Breast and Female Genital Organs. Lyon, France: International Agency for Research on Cancer, 2004.
  4. Bergman L, Beelen ML, Gallee MP, et al.: Risk and prognosis of endometrial cancer after tamoxifen for breast cancer. Comprehensive Cancer Centres' ALERT Group. Assessment of Liver and Endometrial cancer Risk following Tamoxifen. Lancet 356 (9233): 881-7, 2000. [PUBMED Abstract]
  5. Cohen I: Endometrial pathologies associated with postmenopausal tamoxifen treatment. Gynecol Oncol 94 (2): 256-66, 2004. [PUBMED Abstract]
  6. Wickerham DL, Fisher B, Wolmark N, et al.: Association of tamoxifen and uterine sarcoma. J Clin Oncol 20 (11): 2758-60, 2002. [PUBMED Abstract]
  7. Major FJ, Blessing JA, Silverberg SG, et al.: Prognostic factors in early-stage uterine sarcoma. A Gynecologic Oncology Group study. Cancer 71 (4 Suppl): 1702-9, 1993. [PUBMED Abstract]
  8. Evans HL, Chawla SP, Simpson C, et al.: Smooth muscle neoplasms of the uterus other than ordinary leiomyoma. A study of 46 cases, with emphasis on diagnostic criteria and prognostic factors. Cancer 62 (10): 2239-47, 1988. [PUBMED Abstract]
  9. Oláh KS, Dunn JA, Gee H: Leiomyosarcomas have a poorer prognosis than mixed mesodermal tumours when adjusting for known prognostic factors: the result of a retrospective study of 423 cases of uterine sarcoma. Br J Obstet Gynaecol 99 (7): 590-4, 1992. [PUBMED Abstract]
  10. Omura GA, Blessing JA, Major F, et al.: A randomized clinical trial of adjuvant adriamycin in uterine sarcomas: a Gynecologic Oncology Group Study. J Clin Oncol 3 (9): 1240-5, 1985. [PUBMED Abstract]
  11. Piver MS, Lele SB, Marchetti DL, et al.: Effect of adjuvant chemotherapy on time to recurrence and survival of stage I uterine sarcomas. J Surg Oncol 38 (4): 233-9, 1988. [PUBMED Abstract]
  12. van Nagell JR Jr, Hanson MB, Donaldson ES, et al.: Adjuvant vincristine, dactinomycin, and cyclophosphamide therapy in stage I uterine sarcomas. A pilot study. Cancer 57 (8): 1451-4, 1986. [PUBMED Abstract]
  13. Peters WA 3rd, Rivkin SE, Smith MR, et al.: Cisplatin and adriamycin combination chemotherapy for uterine stromal sarcomas and mixed mesodermal tumors. Gynecol Oncol 34 (3): 323-7, 1989. [PUBMED Abstract]

Cellular Classification of Uterine Sarcoma

The most common histologic types of uterine sarcomas include:

  • Carcinosarcomas (mixed mesodermal sarcomas [40%–50%]).
  • Leiomyosarcomas (30%).
  • Endometrial stromal sarcomas (15%).

The uterine neoplasm classification of the International Society of Gynecologic Pathologists and the World Health Organization uses the term carcinosarcomas for all primary uterine neoplasms containing malignant elements of both epithelial and stromal light microscopic appearances, regardless of whether malignant heterologous elements are present.[1]

References
  1. Silverberg SG, Major FJ, Blessing JA, et al.: Carcinosarcoma (malignant mixed mesodermal tumor) of the uterus. A Gynecologic Oncology Group pathologic study of 203 cases. Int J Gynecol Pathol 9 (1): 1-19, 1990. [PUBMED Abstract]

Stage Information for Uterine Sarcoma

Definitions: FIGO

The Féderation Internationale de Gynécologie et d’Obstétrique (FIGO) and the American Joint Committee on Cancer (AJCC) have designated staging to define carcinoma of the corpus uteri, which applies to uterine sarcoma; the FIGO system is most commonly used.[1,2]

Uterine sarcomas include leiomyosarcomas, endometrial stromal sarcomas, and adenosarcomas.

Table 1. Uterine Sarcomaa
StageDescription
aAdapted from FIGO Committee on Gynecologic Oncology.[1]
bEither G1, G2, or G3 (G = grade).
cEndocervical glandular involvement only should be considered as stage I and no longer as stage II.
dPositive cytology has to be reported separately without changing the stage.
IbTumor confined to the corpus uteri.
IAbNo or less than half myometrial invasion.
IBbInvasion equal to or more than half of the myometrium.
IIbTumor invades cervical stroma but does not extend beyond the uterus.c
IIIbLocal and/or regional spread of the tumor.
IIIAbTumor invades the serosa of the corpus uteri and/or adnexae.d
IIIBbVaginal and/or parametrial involvement.d
IIICbMetastases to pelvic and/or para-aortic lymph nodes.d
IIIC1bPositive pelvic nodes.
IIIC2bPositive para-aortic lymph nodes with or without positive pelvic lymph nodes.
IVbTumor invades bladder and/or bowel mucosa, and/or distant metastases.
IVAbTumor invasion of bladder and/or bowel mucosa.
IVBbDistant metastases, including intra-abdominal metastases and/or inguinal lymph nodes.
References
  1. Pecorelli S: Revised FIGO staging for carcinoma of the vulva, cervix, and endometrium. Int J Gynaecol Obstet 105 (2): 103-4, 2009. [PUBMED Abstract]
  2. Corpus uteri. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 403-18.

Treatment Option Overview

Surgery is often the principal means of diagnosis and is the primary treatment for all patients with uterine sarcoma. If the diagnosis is known, the extent of surgery is planned according to the stage of the tumor. Hysterectomy is usually performed when a uterine malignancy is suspected, except for rare instances when preservation of the uterus in a young patient is deemed safe for the type of cancer (e.g., a totally confined low-grade leiomyosarcoma in a woman who desires to retain childbearing potential). Medically suitable patients with the preoperative diagnosis of uterine sarcoma are considered candidates for abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy. Cytologic washings are obtained from the pelvis and abdomen. Thorough examination of the diaphragm, omentum, and upper abdomen is performed.

There is no firm evidence from a prospective study that adjuvant chemotherapy or radiation therapy is of benefit for patients with uterine sarcoma.[1] In one Gynecologic Oncology Group (GOG) study, the use of adjuvant doxorubicin did not alter the survival rate of patients with resected stage I or stage II uterine sarcomas; however, interpretation of these results is difficult because this study included some patients who received radiation and three types of uterine sarcomas that have variable responses to doxorubicin.[1][Level of evidence: 1iiA] However, because the risk of disease recurrence is high even with localized presentations, many physicians have considered the use of adjuvant chemotherapy or radiation therapy.[2] A report of a study (GOG-0150 [NCT00002546]) that addressed radiation therapy versus adjuvant chemotherapy is awaited.[3]

References
  1. Omura GA, Blessing JA, Major F, et al.: A randomized clinical trial of adjuvant adriamycin in uterine sarcomas: a Gynecologic Oncology Group Study. J Clin Oncol 3 (9): 1240-5, 1985. [PUBMED Abstract]
  2. Kohorn EI, Schwartz PE, Chambers JT, et al.: Adjuvant therapy in mixed mullerian tumors of the uterus. Gynecol Oncol 23 (2): 212-21, 1986. [PUBMED Abstract]
  3. Wolfson AH, Brady MF, Mannel RS, et al.: A Gynecologic Oncology Group randomized trial of whole abdominal irradiation (WAI) vs cisplatin-ifosfamide+mesna (CIM) in optimally debulked stage I-IV carcinosarcoma (CS) of the uterus. [Abstract] J Clin Oncol 24 (Suppl 18): A-5001, 256s, 2006.

Stage I Uterine Sarcoma

Standard treatment options:

  1. Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy).
  2. Surgery plus pelvic radiation therapy.
  3. Surgery plus adjuvant chemotherapy.
  4. Surgery plus adjuvant radiation therapy as seen in the EORTC-55874 trial, for example.

In a nonrandomized, Gynecologic Oncology Group study in patients with stage I and II carcinosarcomas, those who had pelvic radiation therapy had a significant reduction of recurrences within the radiation treatment field but no alteration in survival.[1] A large nonrandomized study demonstrated improved survival and a lower local failure rate in patients with mixed mullerian tumors following postoperative external and intracavitary radiation therapy.[2] One nonrandomized study that predominantly included patients with carcinosarcomas appeared to show benefit for adjuvant therapy with cisplatin and doxorubicin.[3]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Hornback NB, Omura G, Major FJ: Observations on the use of adjuvant radiation therapy in patients with stage I and II uterine sarcoma. Int J Radiat Oncol Biol Phys 12 (12): 2127-30, 1986. [PUBMED Abstract]
  2. Larson B, Silfverswärd C, Nilsson B, et al.: Mixed müllerian tumours of the uterus--prognostic factors: a clinical and histopathologic study of 147 cases. Radiother Oncol 17 (2): 123-32, 1990. [PUBMED Abstract]
  3. Peters WA 3rd, Rivkin SE, Smith MR, et al.: Cisplatin and adriamycin combination chemotherapy for uterine stromal sarcomas and mixed mesodermal tumors. Gynecol Oncol 34 (3): 323-7, 1989. [PUBMED Abstract]

Stage II Uterine Sarcoma

Standard treatment options:

  1. Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy).
  2. Surgery plus pelvic radiation therapy.
  3. Surgery plus adjuvant chemotherapy.
  4. Surgery plus adjuvant radiation therapy (EORTC-55874).

In a nonrandomized, Gynecologic Oncology Group study in patients with stage I and II carcinosarcomas, those who had pelvic radiation therapy had a significant reduction of recurrences within the radiation treatment field but no alteration in survival.[1] One nonrandomized study that predominantly included patients with carcinosarcomas appeared to show benefit for adjuvant therapy with cisplatin and doxorubicin.[2]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Hornback NB, Omura G, Major FJ: Observations on the use of adjuvant radiation therapy in patients with stage I and II uterine sarcoma. Int J Radiat Oncol Biol Phys 12 (12): 2127-30, 1986. [PUBMED Abstract]
  2. Peters WA 3rd, Rivkin SE, Smith MR, et al.: Cisplatin and adriamycin combination chemotherapy for uterine stromal sarcomas and mixed mesodermal tumors. Gynecol Oncol 34 (3): 323-7, 1989. [PUBMED Abstract]

Stage III Uterine Sarcoma

Standard treatment options:

  • Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic and periaortic selective lymphadenectomy, and resection of all gross tumor).

Treatment options under clinical evaluation:

  1. Surgery plus pelvic radiation therapy.
  2. Surgery plus adjuvant chemotherapy.

Carcinosarcomas (the preferred designation by the World Health Organization) are also referred to as mixed mesodermal or mullerian tumors. Controversy exists about the following issues:

  • Whether they are true sarcomas.
  • Whether the sarcomatous elements are actually derived from a common epithelial cell precursor that also gives rise to the usually more abundant adenocarcinomatous elements.

The stromal components of the carcinosarcomas are further characterized by whether they contain homologous elements (such as malignant mesenchymal tissue considered possibly native to the uterus) or heterologous elements (such as striated muscle, cartilage, or bone, which are foreign to the uterus). Carcinosarcomas parallel endometrial cancer in its postmenopausal predominance and in other of its epidemiologic features; increasingly, the treatment of carcinosarcomas is becoming similar to combined modality approaches for endometrial adenocarcinomas.

Patients who present with uterine sarcoma have been treated on a series of phase II studies by the Gynecologic Oncology Group, including the GOG-87B trial, for example.[1,2] These chemotherapy studies have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide. These studies have also documented differences in response leading to separate trials for patients with carcinosarcomas and leiomyosarcomas. As an example, in patients previously untreated with chemotherapy, ifosfamide had a 32.2% response rate in patients with carcinosarcomas [3] and a 17.2% partial response rate in patients with leiomyosarcomas.[2]

A randomized comparison that was seen in the GOG-108 trial, for example, of ifosfamide with or without cisplatin for first-line therapy for patients with measurable advanced or recurrent carcinosarcomas demonstrated a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) on the combination arm (6 months vs. 4 months), but there was no significant improvement in survival (9 months vs. 8 months).[4][Level of evidence: 1iiA] The follow-up GOG-0161 [NCT00003128] study utilized 3-day ifosfamide regimens (instead of the more toxic 5-day regimen in the preceding study) for the control and for a combination with paclitaxel (with filgrastim starting on day 4).[5] The combination was superior in response rates (45% vs. 29%), PFS (8.4 months vs. 5.8 months), and overall survival (13.5 months and 8.4 months). The hazard ratio for death favored the combination 0.69 (95% confidence interval, 0.49–0.97).[5][Level of evidence: 1iiA] In this study, 52% of 179 evaluable patients had recurrent disease, 18% had stage III disease, and 30% had stage IV disease. In addition, imbalances were present in the sites of disease and in the use of prior radiation therapy, and 30 patients were excluded for wrong pathology.

A role for chemotherapy as adjuvant to surgery has not yet been established.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Thigpen JT, Blessing JA, Beecham J, et al.: Phase II trial of cisplatin as first-line chemotherapy in patients with advanced or recurrent uterine sarcomas: a Gynecologic Oncology Group study. J Clin Oncol 9 (11): 1962-6, 1991. [PUBMED Abstract]
  2. Sutton GP, Blessing JA, Barrett RJ, et al.: Phase II trial of ifosfamide and mesna in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Am J Obstet Gynecol 166 (2): 556-9, 1992. [PUBMED Abstract]
  3. Sutton GP, Blessing JA, Rosenshein N, et al.: Phase II trial of ifosfamide and mesna in mixed mesodermal tumors of the uterus (a Gynecologic Oncology Group study). Am J Obstet Gynecol 161 (2): 309-12, 1989. [PUBMED Abstract]
  4. Sutton G, Brunetto VL, Kilgore L, et al.: A phase III trial of ifosfamide with or without cisplatin in carcinosarcoma of the uterus: A Gynecologic Oncology Group Study. Gynecol Oncol 79 (2): 147-53, 2000. [PUBMED Abstract]
  5. Homesley HD, Filiaci V, Markman M, et al.: Phase III trial of ifosfamide with or without paclitaxel in advanced uterine carcinosarcoma: a Gynecologic Oncology Group Study. J Clin Oncol 25 (5): 526-31, 2007. [PUBMED Abstract]

Stage IV Uterine Sarcoma

There is currently no standard therapy for patients with stage IV disease. These patients should be entered into an ongoing clinical trial.

Carcinosarcomas (the preferred designation by the World Health Organization) are also referred to as mixed mesodermal or mullerian tumors. Controversy exists about the following issues:

  • Whether they are true sarcomas.
  • Whether the sarcomatous elements are actually derived from a common epithelial cell precursor that also gives rise to the usually more abundant adenocarcinomatous elements.

The stromal components of the carcinosarcomas are further characterized by whether they contain homologous elements, such as malignant mesenchymal tissue considered possibly native to the uterus, or heterologous elements, such as striated muscle, cartilage, or bone, which is foreign to the uterus. Carcinosarcomas parallel endometrial cancer in its postmenopausal predominance and in other of its epidemiologic features; increasingly, the treatment of carcinosarcomas is becoming similar to combined modality approaches for endometrial adenocarcinomas.

Patients who present with uterine sarcoma have been treated on a series of phase II studies by the Gynecologic Oncology Group, including the GOG-87B trial, for example.[1] These chemotherapy studies have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide. These studies have also documented differences in response leading to separate trials for patients with carcinosarcomas and leiomyosarcomas. As an example, in patients previously untreated with chemotherapy, ifosfamide had a 32.2% response rate in patients with carcinosarcomas,[2] a 33% response rate in patients with endometrial stromal cell sarcomas,[3], and a 17.2% partial response rate in patients with leiomyosarcomas.[4] Doxorubicin in combination with dacarbazine or cyclophosphamide is no more active than doxorubicin alone for advanced disease.[5,6] Cisplatin has activity as first-line therapy and minimal activity as second-line therapy for patients with carcinosarcomas, but cisplatin is inactive as first- or second-line therapy for patients with leiomyosarcomas.[1,7]

A randomized comparison that was seen in the GOG-108 trial, for example, of ifosfamide with or without cisplatin for first-line therapy for patients with measurable advanced or recurrent carcinosarcomas demonstrated a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) on the combination arm (6 months vs. 4 months), but there was no significant improvement in survival (9 months vs. 8 months).[8][Level of evidence: 1iiA] The follow-up GOG-0161 [NCT00003128] study utilized 3-day ifosfamide regimens (instead of the more toxic 5-day regimen in the preceding study) for the control and for a combination with paclitaxel (with filgrastim starting on day 4).[9] The combination was superior in response rates (45% vs. 29%), PFS (8.4 months vs. 5.8 months), and overall survival (13.5 months and 8.4 months). The hazard ratio for death favored the combination 0.69 (95% confidence interval, 0.49–0.97).[9][Level of evidence: 1iiA] In this study, 52% of 179 evaluable patients had recurrent disease, 18% had stage III disease, and 30% had stage IV disease. In addition, imbalances were present in the sites of disease and in the use of prior radiation therapy, and 30 patients were excluded for wrong pathology.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Thigpen JT, Blessing JA, Beecham J, et al.: Phase II trial of cisplatin as first-line chemotherapy in patients with advanced or recurrent uterine sarcomas: a Gynecologic Oncology Group study. J Clin Oncol 9 (11): 1962-6, 1991. [PUBMED Abstract]
  2. Sutton GP, Blessing JA, Rosenshein N, et al.: Phase II trial of ifosfamide and mesna in mixed mesodermal tumors of the uterus (a Gynecologic Oncology Group study). Am J Obstet Gynecol 161 (2): 309-12, 1989. [PUBMED Abstract]
  3. Sutton G, Blessing JA, Park R, et al.: Ifosfamide treatment of recurrent or metastatic endometrial stromal sarcomas previously unexposed to chemotherapy: a study of the Gynecologic Oncology Group. Obstet Gynecol 87 (5 Pt 1): 747-50, 1996. [PUBMED Abstract]
  4. Sutton GP, Blessing JA, Barrett RJ, et al.: Phase II trial of ifosfamide and mesna in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Am J Obstet Gynecol 166 (2): 556-9, 1992. [PUBMED Abstract]
  5. Omura GA, Major FJ, Blessing JA, et al.: A randomized study of adriamycin with and without dimethyl triazenoimidazole carboxamide in advanced uterine sarcomas. Cancer 52 (4): 626-32, 1983. [PUBMED Abstract]
  6. Muss HB, Bundy B, DiSaia PJ, et al.: Treatment of recurrent or advanced uterine sarcoma. A randomized trial of doxorubicin versus doxorubicin and cyclophosphamide (a phase III trial of the Gynecologic Oncology Group). Cancer 55 (8): 1648-53, 1985. [PUBMED Abstract]
  7. Thigpen JT, Blessing JA, Wilbanks GD: Cisplatin as second-line chemotherapy in the treatment of advanced or recurrent leiomyosarcoma of the uterus. A phase II trial of the Gynecologic Oncology Group. Am J Clin Oncol 9 (1): 18-20, 1986. [PUBMED Abstract]
  8. Sutton G, Brunetto VL, Kilgore L, et al.: A phase III trial of ifosfamide with or without cisplatin in carcinosarcoma of the uterus: A Gynecologic Oncology Group Study. Gynecol Oncol 79 (2): 147-53, 2000. [PUBMED Abstract]
  9. Homesley HD, Filiaci V, Markman M, et al.: Phase III trial of ifosfamide with or without paclitaxel in advanced uterine carcinosarcoma: a Gynecologic Oncology Group Study. J Clin Oncol 25 (5): 526-31, 2007. [PUBMED Abstract]

Recurrent Uterine Sarcoma

There is currently no standard therapy for patients with recurrent disease. These patients should be entered into an ongoing clinical trial.

Patients who present with uterine sarcoma have been treated on a series of phase II studies by the Gynecologic Oncology Group, including the GOG-87B trial, for example. These chemotherapy studies have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide. These studies have also documented differences in response leading to separate trials for patients with carcinosarcomas and leiomyosarcomas. As an example, in patients previously untreated with chemotherapy, ifosfamide had a 32.2% response rate in patients with carcinosarcomas,[1] a 33% response rate in patients with endometrial stromal cell sarcomas,[2] and a 17.2% partial response rate in patients with leiomyosarcomas.[3] Doxorubicin in combination with dacarbazine or cyclophosphamide is no more active than doxorubicin alone for recurrent disease.[4,5] Cisplatin has activity as first-line therapy and minimal activity as second-line therapy for patients with carcinosarcomas, but cisplatin is inactive as first- or second-line therapy for patients with leiomyosarcomas.[6,7] A regimen of gemcitabine plus docetaxel had a 53% response rate in patients with unresectable leiomyosarcomas and is undergoing further study.[8]

A randomized comparison that was seen in the GOG-108 trial, for example, of ifosfamide with or without cisplatin for first-line therapy for patients with measurable advanced or recurrent carcinosarcomas demonstrated a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) on the combination arm (6 months vs. 4 months), but there was no significant improvement in survival (9 months vs. 8 months).[9][Level of evidence: 1iiA] The follow-up GOG-0161 [NCT00003128] study utilized 3-day ifosfamide regimens (instead of the more toxic 5-day regimen in the preceding study) for the control and for a combination with paclitaxel (with filgrastim starting on day 4).[10] The combination was superior in response rates (45% vs. 29%), PFS (8.4 months vs. 5.8 months), and overall survival (13.5 months and 8.4 months). The hazard ratio for death favored the combination 0.69 (95% confidence interval, 0.49–0.97).[10][Level of evidence: 1iiA] In this study, 52% of 179 evaluable patients had recurrent disease, 18% had stage III disease, and 30% had stage IV disease. In addition, imbalances were present in the sites of disease and in the use of prior radiation therapy, and 30 patients were excluded for wrong pathology.

For patients with carcinosarcomas who have localized recurrence to the pelvis confirmed by computed tomographic scanning, radiation therapy may be effective palliation. Phase I and II clinical trials are appropriate for patients who recur with distant metastasis and are unresponsive to first-line phase II trials. High-dose progesterone hormone therapy may be of some benefit to patients with low-grade stromal sarcoma.[11]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Sutton GP, Blessing JA, Rosenshein N, et al.: Phase II trial of ifosfamide and mesna in mixed mesodermal tumors of the uterus (a Gynecologic Oncology Group study). Am J Obstet Gynecol 161 (2): 309-12, 1989. [PUBMED Abstract]
  2. Sutton G, Blessing JA, Park R, et al.: Ifosfamide treatment of recurrent or metastatic endometrial stromal sarcomas previously unexposed to chemotherapy: a study of the Gynecologic Oncology Group. Obstet Gynecol 87 (5 Pt 1): 747-50, 1996. [PUBMED Abstract]
  3. Sutton GP, Blessing JA, Barrett RJ, et al.: Phase II trial of ifosfamide and mesna in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Am J Obstet Gynecol 166 (2): 556-9, 1992. [PUBMED Abstract]
  4. Omura GA, Major FJ, Blessing JA, et al.: A randomized study of adriamycin with and without dimethyl triazenoimidazole carboxamide in advanced uterine sarcomas. Cancer 52 (4): 626-32, 1983. [PUBMED Abstract]
  5. Muss HB, Bundy B, DiSaia PJ, et al.: Treatment of recurrent or advanced uterine sarcoma. A randomized trial of doxorubicin versus doxorubicin and cyclophosphamide (a phase III trial of the Gynecologic Oncology Group). Cancer 55 (8): 1648-53, 1985. [PUBMED Abstract]
  6. Thigpen JT, Blessing JA, Beecham J, et al.: Phase II trial of cisplatin as first-line chemotherapy in patients with advanced or recurrent uterine sarcomas: a Gynecologic Oncology Group study. J Clin Oncol 9 (11): 1962-6, 1991. [PUBMED Abstract]
  7. Thigpen JT, Blessing JA, Wilbanks GD: Cisplatin as second-line chemotherapy in the treatment of advanced or recurrent leiomyosarcoma of the uterus. A phase II trial of the Gynecologic Oncology Group. Am J Clin Oncol 9 (1): 18-20, 1986. [PUBMED Abstract]
  8. Hensley ML, Maki R, Venkatraman E, et al.: Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol 20 (12): 2824-31, 2002. [PUBMED Abstract]
  9. Sutton G, Brunetto VL, Kilgore L, et al.: A phase III trial of ifosfamide with or without cisplatin in carcinosarcoma of the uterus: A Gynecologic Oncology Group Study. Gynecol Oncol 79 (2): 147-53, 2000. [PUBMED Abstract]
  10. Homesley HD, Filiaci V, Markman M, et al.: Phase III trial of ifosfamide with or without paclitaxel in advanced uterine carcinosarcoma: a Gynecologic Oncology Group Study. J Clin Oncol 25 (5): 526-31, 2007. [PUBMED Abstract]
  11. Katz L, Merino MJ, Sakamoto H, et al.: Endometrial stromal sarcoma: a clinicopathologic study of 11 cases with determination of estrogen and progestin receptor levels in three tumors. Gynecol Oncol 26 (1): 87-97, 1987. [PUBMED Abstract]

Changes to This Summary (07/15/2015)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.

This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

About This PDQ Summary

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of uterine sarcoma. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

  • be discussed at a meeting,
  • be cited with text, or
  • replace or update an existing article that is already cited.

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Uterine Sarcoma Treatment are:

  • Leslie R. Boyd, MD (New York University Medical Center)
  • Franco M. Muggia, MD (New York University Medical Center)

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

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The preferred citation for this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Uterine Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/uterine/hp/uterine-sarcoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389327]

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Uterine sarcoma treatment

Uterine Sarcoma Treatment (PDQ®)–Health Professional Version

General Information About Uterine Sarcoma

Go to Patient Version

Uterine sarcomas comprise less than 1% of gynecologic malignancies and 2% to 5% of all uterine malignancies.[1] The following tumors arise primarily from three distinct tissues:

  1. Carcinosarcomas arising in the endometrium, in other organs of mullerian origin, and accounting for 40% to 50% of all uterine sarcomas.
  2. Leiomyosarcomas arising from myometrial muscle, with a peak incidence occurring at age 50, and accounting for 30% of all uterine sarcomas.
  3. Sarcomas arising in the endometrial stroma, with a peak incidence occurring before menopause for the low-grade tumors and after menopause for the high-grade tumors, and accounting for 15% of all uterine sarcomas.

The three distinct entities are often grouped under uterine sarcomas; however, each type of tumor is currently being studied in separate clinical trials.

Carcinosarcomas (the preferred designation by the World Health Organization [WHO]) are also referred to as mixed mesodermal sarcomas or mullerian tumors. Controversy exists about the following issues:

  • Whether they are true sarcomas.
  • Whether the sarcomatous elements are actually derived from a common epithelial-cell precursor that also gives rise to the usually more abundant adenocarcinomatous elements.

The stromal components of the carcinosarcomas are further characterized by whether they contain homologous elements, such as malignant mesenchymal tissue considered possibly native to the uterus, or heterologous elements, such as striated muscle, cartilage, or bone, which are foreign to the uterus. Carcinosarcomas parallel endometrial cancer in its postmenopausal predominance and in other of its epidemiologic features; increasingly, the treatment of carcinosarcomas is becoming similar to combined modality approaches for endometrial adenocarcinomas.

Other rare forms of uterine sarcomas also fall under the WHO classification of mesenchymal and mixed tumors of the uterus. These include:[2,3]

  • Mixed endometrial stromal and smooth muscle tumors.
  • Adenosarcomas, in which the epithelial elements appear benign within a malignant mesenchymal background.
  • Embryonal botryoides or rhabdomyosarcomas, which are found almost exclusively in infants.
  • PEComa—a perivascular epithelial-cell tumor that may behave in a malignant fashion, which is the latest to be added.

(Refer to the PDQ summary on Childhood Rhabdomyosarcoma for more information.)

Risk Factors

The only documented etiologic factor in 10% to 25% of these malignancies is prior pelvic radiation therapy, which is often administered for benign uterine bleeding that began 5 to 25 years earlier. An increased incidence of uterine sarcoma has been associated with tamoxifen in the treatment of breast cancer. Subsequently, increases have also been noted when tamoxifen was given to prevent breast cancer in women at increased risk—a possible result of the estrogenic effect of tamoxifen on the uterus. Because of this increase, patients on tamoxifen should have follow-up pelvic examinations and should undergo endometrial biopsy if there is any abnormal uterine bleeding.[4-6]

Prognosis

The prognosis for women with uterine sarcoma is primarily dependent on the extent of disease at the time of diagnosis.[7] For women with carcinosarcomas, significant predictors of metastatic disease at initial surgery include:[7]

  • Isthmic or cervical location.
  • Lymphatic vascular space invasion.
  • Serous and clear cell histology.
  • Grade 2 or 3 carcinoma.

The above factors in addition to the following ones correlate with a progression-free interval:[7]

  • Adnexal spread.
  • Lymph node metastases.
  • Tumor size.
  • Peritoneal cytologic findings.
  • Depth of myometrial invasion.

Factors that bear no relationship to the presence or absence of metastases at surgical exploration are:

  • The presence or absence of stromal heterologous elements.
  • The types of such elements.
  • The grade of the stromal components.
  • The mitotic activity of the stromal components.

In one study, women with a well-differentiated sarcomatous component or carcinosarcomas had significantly longer progression-free intervals than those with moderately to poorly differentiated sarcomas for the homologous and heterologous types. The recurrence rate was 44% for homologous tumors and 63% for heterologous tumors. The type of heterologous sarcoma had no effect on the progression-free interval.

For women with leiomyosarcomas, some investigators consider tumor size to be the most important prognostic factor; women with tumors greater than 5.0 cm in maximum diameter have a poor prognosis.[8] However, in a Gynecologic Oncology Group study, the mitotic index was the only factor significantly related to progression-free interval.[7] Leiomyosarcomas matched for other known prognostic factors may be more aggressive than their carcinosarcoma counterparts.[9] The 5-year survival rate for women with stage I disease, which is confined to the corpus, is approximately 50% versus 0% to 20% for the remaining stages.

Surgery alone can be curative if the malignancy is contained within the uterus. The value of pelvic radiation therapy is not established. Current studies consist primarily of phase II chemotherapy trials for patients with advanced disease. Adjuvant chemotherapy following complete resection for patients with stage I or II disease was not established to be effective in a randomized trial.[10] Yet, other nonrandomized trials have reported improved survival following adjuvant chemotherapy with or without radiation therapy.[11-13]

Related Summaries

Other PDQ summaries containing information related to uterine sarcoma include the following:

References
  1. Forney JP, Buschbaum HJ: Classifying, staging, and treating uterine sarcomas. Contemp Ob Gyn 18(3):47, 50, 55-56, 61-62, 64, 69, 1981.
  2. Gershenson D, McGuire W, Gore Martin, et al.: Gynecologic Cancer: Controversies in Management. 3rd ed. New York, NY: Churchill Livingstone, 2004.
  3. Tavassoéli F, Devilee P, et al.: Pathology and Genetics of Tumours of the Breast and Female Genital Organs. Lyon, France: International Agency for Research on Cancer, 2004.
  4. Bergman L, Beelen ML, Gallee MP, et al.: Risk and prognosis of endometrial cancer after tamoxifen for breast cancer. Comprehensive Cancer Centres' ALERT Group. Assessment of Liver and Endometrial cancer Risk following Tamoxifen. Lancet 356 (9233): 881-7, 2000. [PUBMED Abstract]
  5. Cohen I: Endometrial pathologies associated with postmenopausal tamoxifen treatment. Gynecol Oncol 94 (2): 256-66, 2004. [PUBMED Abstract]
  6. Wickerham DL, Fisher B, Wolmark N, et al.: Association of tamoxifen and uterine sarcoma. J Clin Oncol 20 (11): 2758-60, 2002. [PUBMED Abstract]
  7. Major FJ, Blessing JA, Silverberg SG, et al.: Prognostic factors in early-stage uterine sarcoma. A Gynecologic Oncology Group study. Cancer 71 (4 Suppl): 1702-9, 1993. [PUBMED Abstract]
  8. Evans HL, Chawla SP, Simpson C, et al.: Smooth muscle neoplasms of the uterus other than ordinary leiomyoma. A study of 46 cases, with emphasis on diagnostic criteria and prognostic factors. Cancer 62 (10): 2239-47, 1988. [PUBMED Abstract]
  9. Oláh KS, Dunn JA, Gee H: Leiomyosarcomas have a poorer prognosis than mixed mesodermal tumours when adjusting for known prognostic factors: the result of a retrospective study of 423 cases of uterine sarcoma. Br J Obstet Gynaecol 99 (7): 590-4, 1992. [PUBMED Abstract]
  10. Omura GA, Blessing JA, Major F, et al.: A randomized clinical trial of adjuvant adriamycin in uterine sarcomas: a Gynecologic Oncology Group Study. J Clin Oncol 3 (9): 1240-5, 1985. [PUBMED Abstract]
  11. Piver MS, Lele SB, Marchetti DL, et al.: Effect of adjuvant chemotherapy on time to recurrence and survival of stage I uterine sarcomas. J Surg Oncol 38 (4): 233-9, 1988. [PUBMED Abstract]
  12. van Nagell JR Jr, Hanson MB, Donaldson ES, et al.: Adjuvant vincristine, dactinomycin, and cyclophosphamide therapy in stage I uterine sarcomas. A pilot study. Cancer 57 (8): 1451-4, 1986. [PUBMED Abstract]
  13. Peters WA 3rd, Rivkin SE, Smith MR, et al.: Cisplatin and adriamycin combination chemotherapy for uterine stromal sarcomas and mixed mesodermal tumors. Gynecol Oncol 34 (3): 323-7, 1989. [PUBMED Abstract]

Cellular Classification of Uterine Sarcoma

The most common histologic types of uterine sarcomas include:

  • Carcinosarcomas (mixed mesodermal sarcomas [40%–50%]).
  • Leiomyosarcomas (30%).
  • Endometrial stromal sarcomas (15%).

The uterine neoplasm classification of the International Society of Gynecologic Pathologists and the World Health Organization uses the term carcinosarcomas for all primary uterine neoplasms containing malignant elements of both epithelial and stromal light microscopic appearances, regardless of whether malignant heterologous elements are present.[1]

References
  1. Silverberg SG, Major FJ, Blessing JA, et al.: Carcinosarcoma (malignant mixed mesodermal tumor) of the uterus. A Gynecologic Oncology Group pathologic study of 203 cases. Int J Gynecol Pathol 9 (1): 1-19, 1990. [PUBMED Abstract]

Stage Information for Uterine Sarcoma

Definitions: FIGO

The Féderation Internationale de Gynécologie et d’Obstétrique (FIGO) and the American Joint Committee on Cancer (AJCC) have designated staging to define carcinoma of the corpus uteri, which applies to uterine sarcoma; the FIGO system is most commonly used.[1,2]

Uterine sarcomas include leiomyosarcomas, endometrial stromal sarcomas, and adenosarcomas.

Table 1. Uterine Sarcomaa
StageDescription
aAdapted from FIGO Committee on Gynecologic Oncology.[1]
bEither G1, G2, or G3 (G = grade).
cEndocervical glandular involvement only should be considered as stage I and no longer as stage II.
dPositive cytology has to be reported separately without changing the stage.
IbTumor confined to the corpus uteri.
IAbNo or less than half myometrial invasion.
IBbInvasion equal to or more than half of the myometrium.
IIbTumor invades cervical stroma but does not extend beyond the uterus.c
IIIbLocal and/or regional spread of the tumor.
IIIAbTumor invades the serosa of the corpus uteri and/or adnexae.d
IIIBbVaginal and/or parametrial involvement.d
IIICbMetastases to pelvic and/or para-aortic lymph nodes.d
IIIC1bPositive pelvic nodes.
IIIC2bPositive para-aortic lymph nodes with or without positive pelvic lymph nodes.
IVbTumor invades bladder and/or bowel mucosa, and/or distant metastases.
IVAbTumor invasion of bladder and/or bowel mucosa.
IVBbDistant metastases, including intra-abdominal metastases and/or inguinal lymph nodes.
References
  1. Pecorelli S: Revised FIGO staging for carcinoma of the vulva, cervix, and endometrium. Int J Gynaecol Obstet 105 (2): 103-4, 2009. [PUBMED Abstract]
  2. Corpus uteri. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 403-18.

Treatment Option Overview

Surgery is often the principal means of diagnosis and is the primary treatment for all patients with uterine sarcoma. If the diagnosis is known, the extent of surgery is planned according to the stage of the tumor. Hysterectomy is usually performed when a uterine malignancy is suspected, except for rare instances when preservation of the uterus in a young patient is deemed safe for the type of cancer (e.g., a totally confined low-grade leiomyosarcoma in a woman who desires to retain childbearing potential). Medically suitable patients with the preoperative diagnosis of uterine sarcoma are considered candidates for abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy. Cytologic washings are obtained from the pelvis and abdomen. Thorough examination of the diaphragm, omentum, and upper abdomen is performed.

There is no firm evidence from a prospective study that adjuvant chemotherapy or radiation therapy is of benefit for patients with uterine sarcoma.[1] In one Gynecologic Oncology Group (GOG) study, the use of adjuvant doxorubicin did not alter the survival rate of patients with resected stage I or stage II uterine sarcomas; however, interpretation of these results is difficult because this study included some patients who received radiation and three types of uterine sarcomas that have variable responses to doxorubicin.[1][Level of evidence: 1iiA] However, because the risk of disease recurrence is high even with localized presentations, many physicians have considered the use of adjuvant chemotherapy or radiation therapy.[2] A report of a study (GOG-0150 [NCT00002546]) that addressed radiation therapy versus adjuvant chemotherapy is awaited.[3]

References
  1. Omura GA, Blessing JA, Major F, et al.: A randomized clinical trial of adjuvant adriamycin in uterine sarcomas: a Gynecologic Oncology Group Study. J Clin Oncol 3 (9): 1240-5, 1985. [PUBMED Abstract]
  2. Kohorn EI, Schwartz PE, Chambers JT, et al.: Adjuvant therapy in mixed mullerian tumors of the uterus. Gynecol Oncol 23 (2): 212-21, 1986. [PUBMED Abstract]
  3. Wolfson AH, Brady MF, Mannel RS, et al.: A Gynecologic Oncology Group randomized trial of whole abdominal irradiation (WAI) vs cisplatin-ifosfamide+mesna (CIM) in optimally debulked stage I-IV carcinosarcoma (CS) of the uterus. [Abstract] J Clin Oncol 24 (Suppl 18): A-5001, 256s, 2006.

Stage I Uterine Sarcoma

Standard treatment options:

  1. Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy).
  2. Surgery plus pelvic radiation therapy.
  3. Surgery plus adjuvant chemotherapy.
  4. Surgery plus adjuvant radiation therapy as seen in the EORTC-55874 trial, for example.

In a nonrandomized, Gynecologic Oncology Group study in patients with stage I and II carcinosarcomas, those who had pelvic radiation therapy had a significant reduction of recurrences within the radiation treatment field but no alteration in survival.[1] A large nonrandomized study demonstrated improved survival and a lower local failure rate in patients with mixed mullerian tumors following postoperative external and intracavitary radiation therapy.[2] One nonrandomized study that predominantly included patients with carcinosarcomas appeared to show benefit for adjuvant therapy with cisplatin and doxorubicin.[3]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Hornback NB, Omura G, Major FJ: Observations on the use of adjuvant radiation therapy in patients with stage I and II uterine sarcoma. Int J Radiat Oncol Biol Phys 12 (12): 2127-30, 1986. [PUBMED Abstract]
  2. Larson B, Silfverswärd C, Nilsson B, et al.: Mixed müllerian tumours of the uterus--prognostic factors: a clinical and histopathologic study of 147 cases. Radiother Oncol 17 (2): 123-32, 1990. [PUBMED Abstract]
  3. Peters WA 3rd, Rivkin SE, Smith MR, et al.: Cisplatin and adriamycin combination chemotherapy for uterine stromal sarcomas and mixed mesodermal tumors. Gynecol Oncol 34 (3): 323-7, 1989. [PUBMED Abstract]

Stage II Uterine Sarcoma

Standard treatment options:

  1. Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy).
  2. Surgery plus pelvic radiation therapy.
  3. Surgery plus adjuvant chemotherapy.
  4. Surgery plus adjuvant radiation therapy (EORTC-55874).

In a nonrandomized, Gynecologic Oncology Group study in patients with stage I and II carcinosarcomas, those who had pelvic radiation therapy had a significant reduction of recurrences within the radiation treatment field but no alteration in survival.[1] One nonrandomized study that predominantly included patients with carcinosarcomas appeared to show benefit for adjuvant therapy with cisplatin and doxorubicin.[2]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Hornback NB, Omura G, Major FJ: Observations on the use of adjuvant radiation therapy in patients with stage I and II uterine sarcoma. Int J Radiat Oncol Biol Phys 12 (12): 2127-30, 1986. [PUBMED Abstract]
  2. Peters WA 3rd, Rivkin SE, Smith MR, et al.: Cisplatin and adriamycin combination chemotherapy for uterine stromal sarcomas and mixed mesodermal tumors. Gynecol Oncol 34 (3): 323-7, 1989. [PUBMED Abstract]

Stage III Uterine Sarcoma

Standard treatment options:

  • Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic and periaortic selective lymphadenectomy, and resection of all gross tumor).

Treatment options under clinical evaluation:

  1. Surgery plus pelvic radiation therapy.
  2. Surgery plus adjuvant chemotherapy.

Carcinosarcomas (the preferred designation by the World Health Organization) are also referred to as mixed mesodermal or mullerian tumors. Controversy exists about the following issues:

  • Whether they are true sarcomas.
  • Whether the sarcomatous elements are actually derived from a common epithelial cell precursor that also gives rise to the usually more abundant adenocarcinomatous elements.

The stromal components of the carcinosarcomas are further characterized by whether they contain homologous elements (such as malignant mesenchymal tissue considered possibly native to the uterus) or heterologous elements (such as striated muscle, cartilage, or bone, which are foreign to the uterus). Carcinosarcomas parallel endometrial cancer in its postmenopausal predominance and in other of its epidemiologic features; increasingly, the treatment of carcinosarcomas is becoming similar to combined modality approaches for endometrial adenocarcinomas.

Patients who present with uterine sarcoma have been treated on a series of phase II studies by the Gynecologic Oncology Group, including the GOG-87B trial, for example.[1,2] These chemotherapy studies have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide. These studies have also documented differences in response leading to separate trials for patients with carcinosarcomas and leiomyosarcomas. As an example, in patients previously untreated with chemotherapy, ifosfamide had a 32.2% response rate in patients with carcinosarcomas [3] and a 17.2% partial response rate in patients with leiomyosarcomas.[2]

A randomized comparison that was seen in the GOG-108 trial, for example, of ifosfamide with or without cisplatin for first-line therapy for patients with measurable advanced or recurrent carcinosarcomas demonstrated a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) on the combination arm (6 months vs. 4 months), but there was no significant improvement in survival (9 months vs. 8 months).[4][Level of evidence: 1iiA] The follow-up GOG-0161 [NCT00003128] study utilized 3-day ifosfamide regimens (instead of the more toxic 5-day regimen in the preceding study) for the control and for a combination with paclitaxel (with filgrastim starting on day 4).[5] The combination was superior in response rates (45% vs. 29%), PFS (8.4 months vs. 5.8 months), and overall survival (13.5 months and 8.4 months). The hazard ratio for death favored the combination 0.69 (95% confidence interval, 0.49–0.97).[5][Level of evidence: 1iiA] In this study, 52% of 179 evaluable patients had recurrent disease, 18% had stage III disease, and 30% had stage IV disease. In addition, imbalances were present in the sites of disease and in the use of prior radiation therapy, and 30 patients were excluded for wrong pathology.

A role for chemotherapy as adjuvant to surgery has not yet been established.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Thigpen JT, Blessing JA, Beecham J, et al.: Phase II trial of cisplatin as first-line chemotherapy in patients with advanced or recurrent uterine sarcomas: a Gynecologic Oncology Group study. J Clin Oncol 9 (11): 1962-6, 1991. [PUBMED Abstract]
  2. Sutton GP, Blessing JA, Barrett RJ, et al.: Phase II trial of ifosfamide and mesna in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Am J Obstet Gynecol 166 (2): 556-9, 1992. [PUBMED Abstract]
  3. Sutton GP, Blessing JA, Rosenshein N, et al.: Phase II trial of ifosfamide and mesna in mixed mesodermal tumors of the uterus (a Gynecologic Oncology Group study). Am J Obstet Gynecol 161 (2): 309-12, 1989. [PUBMED Abstract]
  4. Sutton G, Brunetto VL, Kilgore L, et al.: A phase III trial of ifosfamide with or without cisplatin in carcinosarcoma of the uterus: A Gynecologic Oncology Group Study. Gynecol Oncol 79 (2): 147-53, 2000. [PUBMED Abstract]
  5. Homesley HD, Filiaci V, Markman M, et al.: Phase III trial of ifosfamide with or without paclitaxel in advanced uterine carcinosarcoma: a Gynecologic Oncology Group Study. J Clin Oncol 25 (5): 526-31, 2007. [PUBMED Abstract]

Stage IV Uterine Sarcoma

There is currently no standard therapy for patients with stage IV disease. These patients should be entered into an ongoing clinical trial.

Carcinosarcomas (the preferred designation by the World Health Organization) are also referred to as mixed mesodermal or mullerian tumors. Controversy exists about the following issues:

  • Whether they are true sarcomas.
  • Whether the sarcomatous elements are actually derived from a common epithelial cell precursor that also gives rise to the usually more abundant adenocarcinomatous elements.

The stromal components of the carcinosarcomas are further characterized by whether they contain homologous elements, such as malignant mesenchymal tissue considered possibly native to the uterus, or heterologous elements, such as striated muscle, cartilage, or bone, which is foreign to the uterus. Carcinosarcomas parallel endometrial cancer in its postmenopausal predominance and in other of its epidemiologic features; increasingly, the treatment of carcinosarcomas is becoming similar to combined modality approaches for endometrial adenocarcinomas.

Patients who present with uterine sarcoma have been treated on a series of phase II studies by the Gynecologic Oncology Group, including the GOG-87B trial, for example.[1] These chemotherapy studies have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide. These studies have also documented differences in response leading to separate trials for patients with carcinosarcomas and leiomyosarcomas. As an example, in patients previously untreated with chemotherapy, ifosfamide had a 32.2% response rate in patients with carcinosarcomas,[2] a 33% response rate in patients with endometrial stromal cell sarcomas,[3], and a 17.2% partial response rate in patients with leiomyosarcomas.[4] Doxorubicin in combination with dacarbazine or cyclophosphamide is no more active than doxorubicin alone for advanced disease.[5,6] Cisplatin has activity as first-line therapy and minimal activity as second-line therapy for patients with carcinosarcomas, but cisplatin is inactive as first- or second-line therapy for patients with leiomyosarcomas.[1,7]

A randomized comparison that was seen in the GOG-108 trial, for example, of ifosfamide with or without cisplatin for first-line therapy for patients with measurable advanced or recurrent carcinosarcomas demonstrated a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) on the combination arm (6 months vs. 4 months), but there was no significant improvement in survival (9 months vs. 8 months).[8][Level of evidence: 1iiA] The follow-up GOG-0161 [NCT00003128] study utilized 3-day ifosfamide regimens (instead of the more toxic 5-day regimen in the preceding study) for the control and for a combination with paclitaxel (with filgrastim starting on day 4).[9] The combination was superior in response rates (45% vs. 29%), PFS (8.4 months vs. 5.8 months), and overall survival (13.5 months and 8.4 months). The hazard ratio for death favored the combination 0.69 (95% confidence interval, 0.49–0.97).[9][Level of evidence: 1iiA] In this study, 52% of 179 evaluable patients had recurrent disease, 18% had stage III disease, and 30% had stage IV disease. In addition, imbalances were present in the sites of disease and in the use of prior radiation therapy, and 30 patients were excluded for wrong pathology.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Thigpen JT, Blessing JA, Beecham J, et al.: Phase II trial of cisplatin as first-line chemotherapy in patients with advanced or recurrent uterine sarcomas: a Gynecologic Oncology Group study. J Clin Oncol 9 (11): 1962-6, 1991. [PUBMED Abstract]
  2. Sutton GP, Blessing JA, Rosenshein N, et al.: Phase II trial of ifosfamide and mesna in mixed mesodermal tumors of the uterus (a Gynecologic Oncology Group study). Am J Obstet Gynecol 161 (2): 309-12, 1989. [PUBMED Abstract]
  3. Sutton G, Blessing JA, Park R, et al.: Ifosfamide treatment of recurrent or metastatic endometrial stromal sarcomas previously unexposed to chemotherapy: a study of the Gynecologic Oncology Group. Obstet Gynecol 87 (5 Pt 1): 747-50, 1996. [PUBMED Abstract]
  4. Sutton GP, Blessing JA, Barrett RJ, et al.: Phase II trial of ifosfamide and mesna in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Am J Obstet Gynecol 166 (2): 556-9, 1992. [PUBMED Abstract]
  5. Omura GA, Major FJ, Blessing JA, et al.: A randomized study of adriamycin with and without dimethyl triazenoimidazole carboxamide in advanced uterine sarcomas. Cancer 52 (4): 626-32, 1983. [PUBMED Abstract]
  6. Muss HB, Bundy B, DiSaia PJ, et al.: Treatment of recurrent or advanced uterine sarcoma. A randomized trial of doxorubicin versus doxorubicin and cyclophosphamide (a phase III trial of the Gynecologic Oncology Group). Cancer 55 (8): 1648-53, 1985. [PUBMED Abstract]
  7. Thigpen JT, Blessing JA, Wilbanks GD: Cisplatin as second-line chemotherapy in the treatment of advanced or recurrent leiomyosarcoma of the uterus. A phase II trial of the Gynecologic Oncology Group. Am J Clin Oncol 9 (1): 18-20, 1986. [PUBMED Abstract]
  8. Sutton G, Brunetto VL, Kilgore L, et al.: A phase III trial of ifosfamide with or without cisplatin in carcinosarcoma of the uterus: A Gynecologic Oncology Group Study. Gynecol Oncol 79 (2): 147-53, 2000. [PUBMED Abstract]
  9. Homesley HD, Filiaci V, Markman M, et al.: Phase III trial of ifosfamide with or without paclitaxel in advanced uterine carcinosarcoma: a Gynecologic Oncology Group Study. J Clin Oncol 25 (5): 526-31, 2007. [PUBMED Abstract]

Recurrent Uterine Sarcoma

There is currently no standard therapy for patients with recurrent disease. These patients should be entered into an ongoing clinical trial.

Patients who present with uterine sarcoma have been treated on a series of phase II studies by the Gynecologic Oncology Group, including the GOG-87B trial, for example. These chemotherapy studies have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide. These studies have also documented differences in response leading to separate trials for patients with carcinosarcomas and leiomyosarcomas. As an example, in patients previously untreated with chemotherapy, ifosfamide had a 32.2% response rate in patients with carcinosarcomas,[1] a 33% response rate in patients with endometrial stromal cell sarcomas,[2] and a 17.2% partial response rate in patients with leiomyosarcomas.[3] Doxorubicin in combination with dacarbazine or cyclophosphamide is no more active than doxorubicin alone for recurrent disease.[4,5] Cisplatin has activity as first-line therapy and minimal activity as second-line therapy for patients with carcinosarcomas, but cisplatin is inactive as first- or second-line therapy for patients with leiomyosarcomas.[6,7] A regimen of gemcitabine plus docetaxel had a 53% response rate in patients with unresectable leiomyosarcomas and is undergoing further study.[8]

A randomized comparison that was seen in the GOG-108 trial, for example, of ifosfamide with or without cisplatin for first-line therapy for patients with measurable advanced or recurrent carcinosarcomas demonstrated a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) on the combination arm (6 months vs. 4 months), but there was no significant improvement in survival (9 months vs. 8 months).[9][Level of evidence: 1iiA] The follow-up GOG-0161 [NCT00003128] study utilized 3-day ifosfamide regimens (instead of the more toxic 5-day regimen in the preceding study) for the control and for a combination with paclitaxel (with filgrastim starting on day 4).[10] The combination was superior in response rates (45% vs. 29%), PFS (8.4 months vs. 5.8 months), and overall survival (13.5 months and 8.4 months). The hazard ratio for death favored the combination 0.69 (95% confidence interval, 0.49–0.97).[10][Level of evidence: 1iiA] In this study, 52% of 179 evaluable patients had recurrent disease, 18% had stage III disease, and 30% had stage IV disease. In addition, imbalances were present in the sites of disease and in the use of prior radiation therapy, and 30 patients were excluded for wrong pathology.

For patients with carcinosarcomas who have localized recurrence to the pelvis confirmed by computed tomographic scanning, radiation therapy may be effective palliation. Phase I and II clinical trials are appropriate for patients who recur with distant metastasis and are unresponsive to first-line phase II trials. High-dose progesterone hormone therapy may be of some benefit to patients with low-grade stromal sarcoma.[11]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Sutton GP, Blessing JA, Rosenshein N, et al.: Phase II trial of ifosfamide and mesna in mixed mesodermal tumors of the uterus (a Gynecologic Oncology Group study). Am J Obstet Gynecol 161 (2): 309-12, 1989. [PUBMED Abstract]
  2. Sutton G, Blessing JA, Park R, et al.: Ifosfamide treatment of recurrent or metastatic endometrial stromal sarcomas previously unexposed to chemotherapy: a study of the Gynecologic Oncology Group. Obstet Gynecol 87 (5 Pt 1): 747-50, 1996. [PUBMED Abstract]
  3. Sutton GP, Blessing JA, Barrett RJ, et al.: Phase II trial of ifosfamide and mesna in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Am J Obstet Gynecol 166 (2): 556-9, 1992. [PUBMED Abstract]
  4. Omura GA, Major FJ, Blessing JA, et al.: A randomized study of adriamycin with and without dimethyl triazenoimidazole carboxamide in advanced uterine sarcomas. Cancer 52 (4): 626-32, 1983. [PUBMED Abstract]
  5. Muss HB, Bundy B, DiSaia PJ, et al.: Treatment of recurrent or advanced uterine sarcoma. A randomized trial of doxorubicin versus doxorubicin and cyclophosphamide (a phase III trial of the Gynecologic Oncology Group). Cancer 55 (8): 1648-53, 1985. [PUBMED Abstract]
  6. Thigpen JT, Blessing JA, Beecham J, et al.: Phase II trial of cisplatin as first-line chemotherapy in patients with advanced or recurrent uterine sarcomas: a Gynecologic Oncology Group study. J Clin Oncol 9 (11): 1962-6, 1991. [PUBMED Abstract]
  7. Thigpen JT, Blessing JA, Wilbanks GD: Cisplatin as second-line chemotherapy in the treatment of advanced or recurrent leiomyosarcoma of the uterus. A phase II trial of the Gynecologic Oncology Group. Am J Clin Oncol 9 (1): 18-20, 1986. [PUBMED Abstract]
  8. Hensley ML, Maki R, Venkatraman E, et al.: Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol 20 (12): 2824-31, 2002. [PUBMED Abstract]
  9. Sutton G, Brunetto VL, Kilgore L, et al.: A phase III trial of ifosfamide with or without cisplatin in carcinosarcoma of the uterus: A Gynecologic Oncology Group Study. Gynecol Oncol 79 (2): 147-53, 2000. [PUBMED Abstract]
  10. Homesley HD, Filiaci V, Markman M, et al.: Phase III trial of ifosfamide with or without paclitaxel in advanced uterine carcinosarcoma: a Gynecologic Oncology Group Study. J Clin Oncol 25 (5): 526-31, 2007. [PUBMED Abstract]
  11. Katz L, Merino MJ, Sakamoto H, et al.: Endometrial stromal sarcoma: a clinicopathologic study of 11 cases with determination of estrogen and progestin receptor levels in three tumors. Gynecol Oncol 26 (1): 87-97, 1987. [PUBMED Abstract]

Changes to This Summary (07/15/2015)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.

This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

About This PDQ Summary

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of uterine sarcoma. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

  • be discussed at a meeting,
  • be cited with text, or
  • replace or update an existing article that is already cited.

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Uterine Sarcoma Treatment are:

  • Leslie R. Boyd, MD (New York University Medical Center)
  • Franco M. Muggia, MD (New York University Medical Center)

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

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The preferred citation for this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Uterine Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/uterine/hp/uterine-sarcoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389327]

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Uterine Sarcoma Treatment (PDQ®)–Health Professional Version

General Information About Uterine Sarcoma

Go to Patient Version

Uterine sarcomas comprise less than 1% of gynecologic malignancies and 2% to 5% of all uterine malignancies.[1] The following tumors arise primarily from three distinct tissues:

  1. Carcinosarcomas arising in the endometrium, in other organs of mullerian origin, and accounting for 40% to 50% of all uterine sarcomas.
  2. Leiomyosarcomas arising from myometrial muscle, with a peak incidence occurring at age 50, and accounting for 30% of all uterine sarcomas.
  3. Sarcomas arising in the endometrial stroma, with a peak incidence occurring before menopause for the low-grade tumors and after menopause for the high-grade tumors, and accounting for 15% of all uterine sarcomas.

The three distinct entities are often grouped under uterine sarcomas; however, each type of tumor is currently being studied in separate clinical trials.

Carcinosarcomas (the preferred designation by the World Health Organization [WHO]) are also referred to as mixed mesodermal sarcomas or mullerian tumors. Controversy exists about the following issues:

  • Whether they are true sarcomas.
  • Whether the sarcomatous elements are actually derived from a common epithelial-cell precursor that also gives rise to the usually more abundant adenocarcinomatous elements.

The stromal components of the carcinosarcomas are further characterized by whether they contain homologous elements, such as malignant mesenchymal tissue considered possibly native to the uterus, or heterologous elements, such as striated muscle, cartilage, or bone, which are foreign to the uterus. Carcinosarcomas parallel endometrial cancer in its postmenopausal predominance and in other of its epidemiologic features; increasingly, the treatment of carcinosarcomas is becoming similar to combined modality approaches for endometrial adenocarcinomas.

Other rare forms of uterine sarcomas also fall under the WHO classification of mesenchymal and mixed tumors of the uterus. These include:[2,3]

  • Mixed endometrial stromal and smooth muscle tumors.
  • Adenosarcomas, in which the epithelial elements appear benign within a malignant mesenchymal background.
  • Embryonal botryoides or rhabdomyosarcomas, which are found almost exclusively in infants.
  • PEComa—a perivascular epithelial-cell tumor that may behave in a malignant fashion, which is the latest to be added.

(Refer to the PDQ summary on Childhood Rhabdomyosarcoma for more information.)

Risk Factors

The only documented etiologic factor in 10% to 25% of these malignancies is prior pelvic radiation therapy, which is often administered for benign uterine bleeding that began 5 to 25 years earlier. An increased incidence of uterine sarcoma has been associated with tamoxifen in the treatment of breast cancer. Subsequently, increases have also been noted when tamoxifen was given to prevent breast cancer in women at increased risk—a possible result of the estrogenic effect of tamoxifen on the uterus. Because of this increase, patients on tamoxifen should have follow-up pelvic examinations and should undergo endometrial biopsy if there is any abnormal uterine bleeding.[4-6]

Prognosis

The prognosis for women with uterine sarcoma is primarily dependent on the extent of disease at the time of diagnosis.[7] For women with carcinosarcomas, significant predictors of metastatic disease at initial surgery include:[7]

  • Isthmic or cervical location.
  • Lymphatic vascular space invasion.
  • Serous and clear cell histology.
  • Grade 2 or 3 carcinoma.

The above factors in addition to the following ones correlate with a progression-free interval:[7]

  • Adnexal spread.
  • Lymph node metastases.
  • Tumor size.
  • Peritoneal cytologic findings.
  • Depth of myometrial invasion.

Factors that bear no relationship to the presence or absence of metastases at surgical exploration are:

  • The presence or absence of stromal heterologous elements.
  • The types of such elements.
  • The grade of the stromal components.
  • The mitotic activity of the stromal components.

In one study, women with a well-differentiated sarcomatous component or carcinosarcomas had significantly longer progression-free intervals than those with moderately to poorly differentiated sarcomas for the homologous and heterologous types. The recurrence rate was 44% for homologous tumors and 63% for heterologous tumors. The type of heterologous sarcoma had no effect on the progression-free interval.

For women with leiomyosarcomas, some investigators consider tumor size to be the most important prognostic factor; women with tumors greater than 5.0 cm in maximum diameter have a poor prognosis.[8] However, in a Gynecologic Oncology Group study, the mitotic index was the only factor significantly related to progression-free interval.[7] Leiomyosarcomas matched for other known prognostic factors may be more aggressive than their carcinosarcoma counterparts.[9] The 5-year survival rate for women with stage I disease, which is confined to the corpus, is approximately 50% versus 0% to 20% for the remaining stages.

Surgery alone can be curative if the malignancy is contained within the uterus. The value of pelvic radiation therapy is not established. Current studies consist primarily of phase II chemotherapy trials for patients with advanced disease. Adjuvant chemotherapy following complete resection for patients with stage I or II disease was not established to be effective in a randomized trial.[10] Yet, other nonrandomized trials have reported improved survival following adjuvant chemotherapy with or without radiation therapy.[11-13]

Related Summaries

Other PDQ summaries containing information related to uterine sarcoma include the following:

References
  1. Forney JP, Buschbaum HJ: Classifying, staging, and treating uterine sarcomas. Contemp Ob Gyn 18(3):47, 50, 55-56, 61-62, 64, 69, 1981.
  2. Gershenson D, McGuire W, Gore Martin, et al.: Gynecologic Cancer: Controversies in Management. 3rd ed. New York, NY: Churchill Livingstone, 2004.
  3. Tavassoéli F, Devilee P, et al.: Pathology and Genetics of Tumours of the Breast and Female Genital Organs. Lyon, France: International Agency for Research on Cancer, 2004.
  4. Bergman L, Beelen ML, Gallee MP, et al.: Risk and prognosis of endometrial cancer after tamoxifen for breast cancer. Comprehensive Cancer Centres' ALERT Group. Assessment of Liver and Endometrial cancer Risk following Tamoxifen. Lancet 356 (9233): 881-7, 2000. [PUBMED Abstract]
  5. Cohen I: Endometrial pathologies associated with postmenopausal tamoxifen treatment. Gynecol Oncol 94 (2): 256-66, 2004. [PUBMED Abstract]
  6. Wickerham DL, Fisher B, Wolmark N, et al.: Association of tamoxifen and uterine sarcoma. J Clin Oncol 20 (11): 2758-60, 2002. [PUBMED Abstract]
  7. Major FJ, Blessing JA, Silverberg SG, et al.: Prognostic factors in early-stage uterine sarcoma. A Gynecologic Oncology Group study. Cancer 71 (4 Suppl): 1702-9, 1993. [PUBMED Abstract]
  8. Evans HL, Chawla SP, Simpson C, et al.: Smooth muscle neoplasms of the uterus other than ordinary leiomyoma. A study of 46 cases, with emphasis on diagnostic criteria and prognostic factors. Cancer 62 (10): 2239-47, 1988. [PUBMED Abstract]
  9. Oláh KS, Dunn JA, Gee H: Leiomyosarcomas have a poorer prognosis than mixed mesodermal tumours when adjusting for known prognostic factors: the result of a retrospective study of 423 cases of uterine sarcoma. Br J Obstet Gynaecol 99 (7): 590-4, 1992. [PUBMED Abstract]
  10. Omura GA, Blessing JA, Major F, et al.: A randomized clinical trial of adjuvant adriamycin in uterine sarcomas: a Gynecologic Oncology Group Study. J Clin Oncol 3 (9): 1240-5, 1985. [PUBMED Abstract]
  11. Piver MS, Lele SB, Marchetti DL, et al.: Effect of adjuvant chemotherapy on time to recurrence and survival of stage I uterine sarcomas. J Surg Oncol 38 (4): 233-9, 1988. [PUBMED Abstract]
  12. van Nagell JR Jr, Hanson MB, Donaldson ES, et al.: Adjuvant vincristine, dactinomycin, and cyclophosphamide therapy in stage I uterine sarcomas. A pilot study. Cancer 57 (8): 1451-4, 1986. [PUBMED Abstract]
  13. Peters WA 3rd, Rivkin SE, Smith MR, et al.: Cisplatin and adriamycin combination chemotherapy for uterine stromal sarcomas and mixed mesodermal tumors. Gynecol Oncol 34 (3): 323-7, 1989. [PUBMED Abstract]

Cellular Classification of Uterine Sarcoma

The most common histologic types of uterine sarcomas include:

  • Carcinosarcomas (mixed mesodermal sarcomas [40%–50%]).
  • Leiomyosarcomas (30%).
  • Endometrial stromal sarcomas (15%).

The uterine neoplasm classification of the International Society of Gynecologic Pathologists and the World Health Organization uses the term carcinosarcomas for all primary uterine neoplasms containing malignant elements of both epithelial and stromal light microscopic appearances, regardless of whether malignant heterologous elements are present.[1]

References
  1. Silverberg SG, Major FJ, Blessing JA, et al.: Carcinosarcoma (malignant mixed mesodermal tumor) of the uterus. A Gynecologic Oncology Group pathologic study of 203 cases. Int J Gynecol Pathol 9 (1): 1-19, 1990. [PUBMED Abstract]

Stage Information for Uterine Sarcoma

Definitions: FIGO

The Féderation Internationale de Gynécologie et d’Obstétrique (FIGO) and the American Joint Committee on Cancer (AJCC) have designated staging to define carcinoma of the corpus uteri, which applies to uterine sarcoma; the FIGO system is most commonly used.[1,2]

Uterine sarcomas include leiomyosarcomas, endometrial stromal sarcomas, and adenosarcomas.

Table 1. Uterine Sarcomaa
StageDescription
aAdapted from FIGO Committee on Gynecologic Oncology.[1]
bEither G1, G2, or G3 (G = grade).
cEndocervical glandular involvement only should be considered as stage I and no longer as stage II.
dPositive cytology has to be reported separately without changing the stage.
IbTumor confined to the corpus uteri.
IAbNo or less than half myometrial invasion.
IBbInvasion equal to or more than half of the myometrium.
IIbTumor invades cervical stroma but does not extend beyond the uterus.c
IIIbLocal and/or regional spread of the tumor.
IIIAbTumor invades the serosa of the corpus uteri and/or adnexae.d
IIIBbVaginal and/or parametrial involvement.d
IIICbMetastases to pelvic and/or para-aortic lymph nodes.d
IIIC1bPositive pelvic nodes.
IIIC2bPositive para-aortic lymph nodes with or without positive pelvic lymph nodes.
IVbTumor invades bladder and/or bowel mucosa, and/or distant metastases.
IVAbTumor invasion of bladder and/or bowel mucosa.
IVBbDistant metastases, including intra-abdominal metastases and/or inguinal lymph nodes.
References
  1. Pecorelli S: Revised FIGO staging for carcinoma of the vulva, cervix, and endometrium. Int J Gynaecol Obstet 105 (2): 103-4, 2009. [PUBMED Abstract]
  2. Corpus uteri. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 403-18.

Treatment Option Overview

Surgery is often the principal means of diagnosis and is the primary treatment for all patients with uterine sarcoma. If the diagnosis is known, the extent of surgery is planned according to the stage of the tumor. Hysterectomy is usually performed when a uterine malignancy is suspected, except for rare instances when preservation of the uterus in a young patient is deemed safe for the type of cancer (e.g., a totally confined low-grade leiomyosarcoma in a woman who desires to retain childbearing potential). Medically suitable patients with the preoperative diagnosis of uterine sarcoma are considered candidates for abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy. Cytologic washings are obtained from the pelvis and abdomen. Thorough examination of the diaphragm, omentum, and upper abdomen is performed.

There is no firm evidence from a prospective study that adjuvant chemotherapy or radiation therapy is of benefit for patients with uterine sarcoma.[1] In one Gynecologic Oncology Group (GOG) study, the use of adjuvant doxorubicin did not alter the survival rate of patients with resected stage I or stage II uterine sarcomas; however, interpretation of these results is difficult because this study included some patients who received radiation and three types of uterine sarcomas that have variable responses to doxorubicin.[1][Level of evidence: 1iiA] However, because the risk of disease recurrence is high even with localized presentations, many physicians have considered the use of adjuvant chemotherapy or radiation therapy.[2] A report of a study (GOG-0150 [NCT00002546]) that addressed radiation therapy versus adjuvant chemotherapy is awaited.[3]

References
  1. Omura GA, Blessing JA, Major F, et al.: A randomized clinical trial of adjuvant adriamycin in uterine sarcomas: a Gynecologic Oncology Group Study. J Clin Oncol 3 (9): 1240-5, 1985. [PUBMED Abstract]
  2. Kohorn EI, Schwartz PE, Chambers JT, et al.: Adjuvant therapy in mixed mullerian tumors of the uterus. Gynecol Oncol 23 (2): 212-21, 1986. [PUBMED Abstract]
  3. Wolfson AH, Brady MF, Mannel RS, et al.: A Gynecologic Oncology Group randomized trial of whole abdominal irradiation (WAI) vs cisplatin-ifosfamide+mesna (CIM) in optimally debulked stage I-IV carcinosarcoma (CS) of the uterus. [Abstract] J Clin Oncol 24 (Suppl 18): A-5001, 256s, 2006.

Stage I Uterine Sarcoma

Standard treatment options:

  1. Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy).
  2. Surgery plus pelvic radiation therapy.
  3. Surgery plus adjuvant chemotherapy.
  4. Surgery plus adjuvant radiation therapy as seen in the EORTC-55874 trial, for example.

In a nonrandomized, Gynecologic Oncology Group study in patients with stage I and II carcinosarcomas, those who had pelvic radiation therapy had a significant reduction of recurrences within the radiation treatment field but no alteration in survival.[1] A large nonrandomized study demonstrated improved survival and a lower local failure rate in patients with mixed mullerian tumors following postoperative external and intracavitary radiation therapy.[2] One nonrandomized study that predominantly included patients with carcinosarcomas appeared to show benefit for adjuvant therapy with cisplatin and doxorubicin.[3]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Hornback NB, Omura G, Major FJ: Observations on the use of adjuvant radiation therapy in patients with stage I and II uterine sarcoma. Int J Radiat Oncol Biol Phys 12 (12): 2127-30, 1986. [PUBMED Abstract]
  2. Larson B, Silfverswärd C, Nilsson B, et al.: Mixed müllerian tumours of the uterus--prognostic factors: a clinical and histopathologic study of 147 cases. Radiother Oncol 17 (2): 123-32, 1990. [PUBMED Abstract]
  3. Peters WA 3rd, Rivkin SE, Smith MR, et al.: Cisplatin and adriamycin combination chemotherapy for uterine stromal sarcomas and mixed mesodermal tumors. Gynecol Oncol 34 (3): 323-7, 1989. [PUBMED Abstract]

Stage II Uterine Sarcoma

Standard treatment options:

  1. Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic and periaortic selective lymphadenectomy).
  2. Surgery plus pelvic radiation therapy.
  3. Surgery plus adjuvant chemotherapy.
  4. Surgery plus adjuvant radiation therapy (EORTC-55874).

In a nonrandomized, Gynecologic Oncology Group study in patients with stage I and II carcinosarcomas, those who had pelvic radiation therapy had a significant reduction of recurrences within the radiation treatment field but no alteration in survival.[1] One nonrandomized study that predominantly included patients with carcinosarcomas appeared to show benefit for adjuvant therapy with cisplatin and doxorubicin.[2]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Hornback NB, Omura G, Major FJ: Observations on the use of adjuvant radiation therapy in patients with stage I and II uterine sarcoma. Int J Radiat Oncol Biol Phys 12 (12): 2127-30, 1986. [PUBMED Abstract]
  2. Peters WA 3rd, Rivkin SE, Smith MR, et al.: Cisplatin and adriamycin combination chemotherapy for uterine stromal sarcomas and mixed mesodermal tumors. Gynecol Oncol 34 (3): 323-7, 1989. [PUBMED Abstract]

Stage III Uterine Sarcoma

Standard treatment options:

  • Surgery (total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic and periaortic selective lymphadenectomy, and resection of all gross tumor).

Treatment options under clinical evaluation:

  1. Surgery plus pelvic radiation therapy.
  2. Surgery plus adjuvant chemotherapy.

Carcinosarcomas (the preferred designation by the World Health Organization) are also referred to as mixed mesodermal or mullerian tumors. Controversy exists about the following issues:

  • Whether they are true sarcomas.
  • Whether the sarcomatous elements are actually derived from a common epithelial cell precursor that also gives rise to the usually more abundant adenocarcinomatous elements.

The stromal components of the carcinosarcomas are further characterized by whether they contain homologous elements (such as malignant mesenchymal tissue considered possibly native to the uterus) or heterologous elements (such as striated muscle, cartilage, or bone, which are foreign to the uterus). Carcinosarcomas parallel endometrial cancer in its postmenopausal predominance and in other of its epidemiologic features; increasingly, the treatment of carcinosarcomas is becoming similar to combined modality approaches for endometrial adenocarcinomas.

Patients who present with uterine sarcoma have been treated on a series of phase II studies by the Gynecologic Oncology Group, including the GOG-87B trial, for example.[1,2] These chemotherapy studies have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide. These studies have also documented differences in response leading to separate trials for patients with carcinosarcomas and leiomyosarcomas. As an example, in patients previously untreated with chemotherapy, ifosfamide had a 32.2% response rate in patients with carcinosarcomas [3] and a 17.2% partial response rate in patients with leiomyosarcomas.[2]

A randomized comparison that was seen in the GOG-108 trial, for example, of ifosfamide with or without cisplatin for first-line therapy for patients with measurable advanced or recurrent carcinosarcomas demonstrated a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) on the combination arm (6 months vs. 4 months), but there was no significant improvement in survival (9 months vs. 8 months).[4][Level of evidence: 1iiA] The follow-up GOG-0161 [NCT00003128] study utilized 3-day ifosfamide regimens (instead of the more toxic 5-day regimen in the preceding study) for the control and for a combination with paclitaxel (with filgrastim starting on day 4).[5] The combination was superior in response rates (45% vs. 29%), PFS (8.4 months vs. 5.8 months), and overall survival (13.5 months and 8.4 months). The hazard ratio for death favored the combination 0.69 (95% confidence interval, 0.49–0.97).[5][Level of evidence: 1iiA] In this study, 52% of 179 evaluable patients had recurrent disease, 18% had stage III disease, and 30% had stage IV disease. In addition, imbalances were present in the sites of disease and in the use of prior radiation therapy, and 30 patients were excluded for wrong pathology.

A role for chemotherapy as adjuvant to surgery has not yet been established.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Thigpen JT, Blessing JA, Beecham J, et al.: Phase II trial of cisplatin as first-line chemotherapy in patients with advanced or recurrent uterine sarcomas: a Gynecologic Oncology Group study. J Clin Oncol 9 (11): 1962-6, 1991. [PUBMED Abstract]
  2. Sutton GP, Blessing JA, Barrett RJ, et al.: Phase II trial of ifosfamide and mesna in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Am J Obstet Gynecol 166 (2): 556-9, 1992. [PUBMED Abstract]
  3. Sutton GP, Blessing JA, Rosenshein N, et al.: Phase II trial of ifosfamide and mesna in mixed mesodermal tumors of the uterus (a Gynecologic Oncology Group study). Am J Obstet Gynecol 161 (2): 309-12, 1989. [PUBMED Abstract]
  4. Sutton G, Brunetto VL, Kilgore L, et al.: A phase III trial of ifosfamide with or without cisplatin in carcinosarcoma of the uterus: A Gynecologic Oncology Group Study. Gynecol Oncol 79 (2): 147-53, 2000. [PUBMED Abstract]
  5. Homesley HD, Filiaci V, Markman M, et al.: Phase III trial of ifosfamide with or without paclitaxel in advanced uterine carcinosarcoma: a Gynecologic Oncology Group Study. J Clin Oncol 25 (5): 526-31, 2007. [PUBMED Abstract]

Stage IV Uterine Sarcoma

There is currently no standard therapy for patients with stage IV disease. These patients should be entered into an ongoing clinical trial.

Carcinosarcomas (the preferred designation by the World Health Organization) are also referred to as mixed mesodermal or mullerian tumors. Controversy exists about the following issues:

  • Whether they are true sarcomas.
  • Whether the sarcomatous elements are actually derived from a common epithelial cell precursor that also gives rise to the usually more abundant adenocarcinomatous elements.

The stromal components of the carcinosarcomas are further characterized by whether they contain homologous elements, such as malignant mesenchymal tissue considered possibly native to the uterus, or heterologous elements, such as striated muscle, cartilage, or bone, which is foreign to the uterus. Carcinosarcomas parallel endometrial cancer in its postmenopausal predominance and in other of its epidemiologic features; increasingly, the treatment of carcinosarcomas is becoming similar to combined modality approaches for endometrial adenocarcinomas.

Patients who present with uterine sarcoma have been treated on a series of phase II studies by the Gynecologic Oncology Group, including the GOG-87B trial, for example.[1] These chemotherapy studies have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide. These studies have also documented differences in response leading to separate trials for patients with carcinosarcomas and leiomyosarcomas. As an example, in patients previously untreated with chemotherapy, ifosfamide had a 32.2% response rate in patients with carcinosarcomas,[2] a 33% response rate in patients with endometrial stromal cell sarcomas,[3], and a 17.2% partial response rate in patients with leiomyosarcomas.[4] Doxorubicin in combination with dacarbazine or cyclophosphamide is no more active than doxorubicin alone for advanced disease.[5,6] Cisplatin has activity as first-line therapy and minimal activity as second-line therapy for patients with carcinosarcomas, but cisplatin is inactive as first- or second-line therapy for patients with leiomyosarcomas.[1,7]

A randomized comparison that was seen in the GOG-108 trial, for example, of ifosfamide with or without cisplatin for first-line therapy for patients with measurable advanced or recurrent carcinosarcomas demonstrated a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) on the combination arm (6 months vs. 4 months), but there was no significant improvement in survival (9 months vs. 8 months).[8][Level of evidence: 1iiA] The follow-up GOG-0161 [NCT00003128] study utilized 3-day ifosfamide regimens (instead of the more toxic 5-day regimen in the preceding study) for the control and for a combination with paclitaxel (with filgrastim starting on day 4).[9] The combination was superior in response rates (45% vs. 29%), PFS (8.4 months vs. 5.8 months), and overall survival (13.5 months and 8.4 months). The hazard ratio for death favored the combination 0.69 (95% confidence interval, 0.49–0.97).[9][Level of evidence: 1iiA] In this study, 52% of 179 evaluable patients had recurrent disease, 18% had stage III disease, and 30% had stage IV disease. In addition, imbalances were present in the sites of disease and in the use of prior radiation therapy, and 30 patients were excluded for wrong pathology.

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Thigpen JT, Blessing JA, Beecham J, et al.: Phase II trial of cisplatin as first-line chemotherapy in patients with advanced or recurrent uterine sarcomas: a Gynecologic Oncology Group study. J Clin Oncol 9 (11): 1962-6, 1991. [PUBMED Abstract]
  2. Sutton GP, Blessing JA, Rosenshein N, et al.: Phase II trial of ifosfamide and mesna in mixed mesodermal tumors of the uterus (a Gynecologic Oncology Group study). Am J Obstet Gynecol 161 (2): 309-12, 1989. [PUBMED Abstract]
  3. Sutton G, Blessing JA, Park R, et al.: Ifosfamide treatment of recurrent or metastatic endometrial stromal sarcomas previously unexposed to chemotherapy: a study of the Gynecologic Oncology Group. Obstet Gynecol 87 (5 Pt 1): 747-50, 1996. [PUBMED Abstract]
  4. Sutton GP, Blessing JA, Barrett RJ, et al.: Phase II trial of ifosfamide and mesna in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Am J Obstet Gynecol 166 (2): 556-9, 1992. [PUBMED Abstract]
  5. Omura GA, Major FJ, Blessing JA, et al.: A randomized study of adriamycin with and without dimethyl triazenoimidazole carboxamide in advanced uterine sarcomas. Cancer 52 (4): 626-32, 1983. [PUBMED Abstract]
  6. Muss HB, Bundy B, DiSaia PJ, et al.: Treatment of recurrent or advanced uterine sarcoma. A randomized trial of doxorubicin versus doxorubicin and cyclophosphamide (a phase III trial of the Gynecologic Oncology Group). Cancer 55 (8): 1648-53, 1985. [PUBMED Abstract]
  7. Thigpen JT, Blessing JA, Wilbanks GD: Cisplatin as second-line chemotherapy in the treatment of advanced or recurrent leiomyosarcoma of the uterus. A phase II trial of the Gynecologic Oncology Group. Am J Clin Oncol 9 (1): 18-20, 1986. [PUBMED Abstract]
  8. Sutton G, Brunetto VL, Kilgore L, et al.: A phase III trial of ifosfamide with or without cisplatin in carcinosarcoma of the uterus: A Gynecologic Oncology Group Study. Gynecol Oncol 79 (2): 147-53, 2000. [PUBMED Abstract]
  9. Homesley HD, Filiaci V, Markman M, et al.: Phase III trial of ifosfamide with or without paclitaxel in advanced uterine carcinosarcoma: a Gynecologic Oncology Group Study. J Clin Oncol 25 (5): 526-31, 2007. [PUBMED Abstract]

Recurrent Uterine Sarcoma

There is currently no standard therapy for patients with recurrent disease. These patients should be entered into an ongoing clinical trial.

Patients who present with uterine sarcoma have been treated on a series of phase II studies by the Gynecologic Oncology Group, including the GOG-87B trial, for example. These chemotherapy studies have documented some antitumor activity for cisplatin, doxorubicin, and ifosfamide. These studies have also documented differences in response leading to separate trials for patients with carcinosarcomas and leiomyosarcomas. As an example, in patients previously untreated with chemotherapy, ifosfamide had a 32.2% response rate in patients with carcinosarcomas,[1] a 33% response rate in patients with endometrial stromal cell sarcomas,[2] and a 17.2% partial response rate in patients with leiomyosarcomas.[3] Doxorubicin in combination with dacarbazine or cyclophosphamide is no more active than doxorubicin alone for recurrent disease.[4,5] Cisplatin has activity as first-line therapy and minimal activity as second-line therapy for patients with carcinosarcomas, but cisplatin is inactive as first- or second-line therapy for patients with leiomyosarcomas.[6,7] A regimen of gemcitabine plus docetaxel had a 53% response rate in patients with unresectable leiomyosarcomas and is undergoing further study.[8]

A randomized comparison that was seen in the GOG-108 trial, for example, of ifosfamide with or without cisplatin for first-line therapy for patients with measurable advanced or recurrent carcinosarcomas demonstrated a higher response rate (54% vs. 34%) and longer progression-free survival (PFS) on the combination arm (6 months vs. 4 months), but there was no significant improvement in survival (9 months vs. 8 months).[9][Level of evidence: 1iiA] The follow-up GOG-0161 [NCT00003128] study utilized 3-day ifosfamide regimens (instead of the more toxic 5-day regimen in the preceding study) for the control and for a combination with paclitaxel (with filgrastim starting on day 4).[10] The combination was superior in response rates (45% vs. 29%), PFS (8.4 months vs. 5.8 months), and overall survival (13.5 months and 8.4 months). The hazard ratio for death favored the combination 0.69 (95% confidence interval, 0.49–0.97).[10][Level of evidence: 1iiA] In this study, 52% of 179 evaluable patients had recurrent disease, 18% had stage III disease, and 30% had stage IV disease. In addition, imbalances were present in the sites of disease and in the use of prior radiation therapy, and 30 patients were excluded for wrong pathology.

For patients with carcinosarcomas who have localized recurrence to the pelvis confirmed by computed tomographic scanning, radiation therapy may be effective palliation. Phase I and II clinical trials are appropriate for patients who recur with distant metastasis and are unresponsive to first-line phase II trials. High-dose progesterone hormone therapy may be of some benefit to patients with low-grade stromal sarcoma.[11]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.

References
  1. Sutton GP, Blessing JA, Rosenshein N, et al.: Phase II trial of ifosfamide and mesna in mixed mesodermal tumors of the uterus (a Gynecologic Oncology Group study). Am J Obstet Gynecol 161 (2): 309-12, 1989. [PUBMED Abstract]
  2. Sutton G, Blessing JA, Park R, et al.: Ifosfamide treatment of recurrent or metastatic endometrial stromal sarcomas previously unexposed to chemotherapy: a study of the Gynecologic Oncology Group. Obstet Gynecol 87 (5 Pt 1): 747-50, 1996. [PUBMED Abstract]
  3. Sutton GP, Blessing JA, Barrett RJ, et al.: Phase II trial of ifosfamide and mesna in leiomyosarcoma of the uterus: a Gynecologic Oncology Group study. Am J Obstet Gynecol 166 (2): 556-9, 1992. [PUBMED Abstract]
  4. Omura GA, Major FJ, Blessing JA, et al.: A randomized study of adriamycin with and without dimethyl triazenoimidazole carboxamide in advanced uterine sarcomas. Cancer 52 (4): 626-32, 1983. [PUBMED Abstract]
  5. Muss HB, Bundy B, DiSaia PJ, et al.: Treatment of recurrent or advanced uterine sarcoma. A randomized trial of doxorubicin versus doxorubicin and cyclophosphamide (a phase III trial of the Gynecologic Oncology Group). Cancer 55 (8): 1648-53, 1985. [PUBMED Abstract]
  6. Thigpen JT, Blessing JA, Beecham J, et al.: Phase II trial of cisplatin as first-line chemotherapy in patients with advanced or recurrent uterine sarcomas: a Gynecologic Oncology Group study. J Clin Oncol 9 (11): 1962-6, 1991. [PUBMED Abstract]
  7. Thigpen JT, Blessing JA, Wilbanks GD: Cisplatin as second-line chemotherapy in the treatment of advanced or recurrent leiomyosarcoma of the uterus. A phase II trial of the Gynecologic Oncology Group. Am J Clin Oncol 9 (1): 18-20, 1986. [PUBMED Abstract]
  8. Hensley ML, Maki R, Venkatraman E, et al.: Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol 20 (12): 2824-31, 2002. [PUBMED Abstract]
  9. Sutton G, Brunetto VL, Kilgore L, et al.: A phase III trial of ifosfamide with or without cisplatin in carcinosarcoma of the uterus: A Gynecologic Oncology Group Study. Gynecol Oncol 79 (2): 147-53, 2000. [PUBMED Abstract]
  10. Homesley HD, Filiaci V, Markman M, et al.: Phase III trial of ifosfamide with or without paclitaxel in advanced uterine carcinosarcoma: a Gynecologic Oncology Group Study. J Clin Oncol 25 (5): 526-31, 2007. [PUBMED Abstract]
  11. Katz L, Merino MJ, Sakamoto H, et al.: Endometrial stromal sarcoma: a clinicopathologic study of 11 cases with determination of estrogen and progestin receptor levels in three tumors. Gynecol Oncol 26 (1): 87-97, 1987. [PUBMED Abstract]

Changes to This Summary (07/15/2015)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Editorial changes were made to this summary.

This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

About This PDQ Summary

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of uterine sarcoma. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

  • be discussed at a meeting,
  • be cited with text, or
  • replace or update an existing article that is already cited.

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Uterine Sarcoma Treatment are:

  • Leslie R. Boyd, MD (New York University Medical Center)
  • Franco M. Muggia, MD (New York University Medical Center)

Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

Permission to Use This Summary

PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”

The preferred citation for this PDQ summary is:

PDQ® Adult Treatment Editorial Board. PDQ Uterine Sarcoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/uterine/hp/uterine-sarcoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389327]

Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.

Disclaimer

Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

Contact Us

More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s Email Us.

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SFA's Medical Advisory Board

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The board is headed by leading US sarcoma experts

The Medical Advisory Board of the Sarcoma Foundation of America includes the following leading oncologist authorities on sarcomas. More information are their treatment centers can be found at https://www.curesarcoma.org/patient-resources/sarcoma-treatment-centers/

Co-Chair: George Demetri, MD, Dana-Farber Cancer Institute
Co-Chair: Raphael Pollock, MD, PhD, The Ohio State University
Mark Agulnik, MD, FRCPC, Robert H. Lurie Comprehensive Cancer Center
Charles Forscher, MD, Cedars-Sinai Medical Center
John M. Goldberg, MD, H3 Biomedicine
Richard Gorlick, MD, MD Anderson Cancer Center
Lee Helman, MD, University of Southern California/Children’s Hospital Los Angeles
Eugenie S. Kleinerman, MD, MD Anderson Cancer Center
Andrew S. Kraft, MD, University of Arizona Cancer Center
Marc Ladanyi, MD, Memorial Sloan-Kettering Cancer Center
David M. Loeb, MD, PhD, Children’s Hospital at Montefiore
Crystal Mackall, MD, Stanford Cancer Institute
Robert Maki, MD, PhD, Cold Spring Harbor Laboratory
Paul A. Meyers, MD, Memorial Sloan-Kettering Cancer Center
Alberto Pappo, MD, St. Jude Children’s Research Hospital
Shreyaskumar Patel, MD, MD Anderson Cancer Center
Peter Pisters, MD, University Health Network
R. Lor Randall, MD, FACS, Huntsman Cancer Center, University of Utah
Richard F. Riedel, MD, Duke University Medical Center
Scott Schuetze, MD, University of Michigan
Samuel Singer, MD, Memorial Sloan-Kettering Cancer Center
Poul HB Sorensen, MD, PhD, University of British Columbia
William D. Tap, MD, Memorial Sloan-Kettering Cancer Center
Jonathan Trent, MD, PhD, Sylvester Comprehensive Cancer Center
Matt van de Rijn, MD, Stanford School of Medicine

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The Sarcoma Journal
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From the Sarcoma Foundation of America
The board is headed by leading US sarcoma experts
The board is headed by leading US sarcoma experts

The Medical Advisory Board of the Sarcoma Foundation of America includes the following leading oncologist authorities on sarcomas. More information are their treatment centers can be found at https://www.curesarcoma.org/patient-resources/sarcoma-treatment-centers/

Co-Chair: George Demetri, MD, Dana-Farber Cancer Institute
Co-Chair: Raphael Pollock, MD, PhD, The Ohio State University
Mark Agulnik, MD, FRCPC, Robert H. Lurie Comprehensive Cancer Center
Charles Forscher, MD, Cedars-Sinai Medical Center
John M. Goldberg, MD, H3 Biomedicine
Richard Gorlick, MD, MD Anderson Cancer Center
Lee Helman, MD, University of Southern California/Children’s Hospital Los Angeles
Eugenie S. Kleinerman, MD, MD Anderson Cancer Center
Andrew S. Kraft, MD, University of Arizona Cancer Center
Marc Ladanyi, MD, Memorial Sloan-Kettering Cancer Center
David M. Loeb, MD, PhD, Children’s Hospital at Montefiore
Crystal Mackall, MD, Stanford Cancer Institute
Robert Maki, MD, PhD, Cold Spring Harbor Laboratory
Paul A. Meyers, MD, Memorial Sloan-Kettering Cancer Center
Alberto Pappo, MD, St. Jude Children’s Research Hospital
Shreyaskumar Patel, MD, MD Anderson Cancer Center
Peter Pisters, MD, University Health Network
R. Lor Randall, MD, FACS, Huntsman Cancer Center, University of Utah
Richard F. Riedel, MD, Duke University Medical Center
Scott Schuetze, MD, University of Michigan
Samuel Singer, MD, Memorial Sloan-Kettering Cancer Center
Poul HB Sorensen, MD, PhD, University of British Columbia
William D. Tap, MD, Memorial Sloan-Kettering Cancer Center
Jonathan Trent, MD, PhD, Sylvester Comprehensive Cancer Center
Matt van de Rijn, MD, Stanford School of Medicine

The Medical Advisory Board of the Sarcoma Foundation of America includes the following leading oncologist authorities on sarcomas. More information are their treatment centers can be found at https://www.curesarcoma.org/patient-resources/sarcoma-treatment-centers/

Co-Chair: George Demetri, MD, Dana-Farber Cancer Institute
Co-Chair: Raphael Pollock, MD, PhD, The Ohio State University
Mark Agulnik, MD, FRCPC, Robert H. Lurie Comprehensive Cancer Center
Charles Forscher, MD, Cedars-Sinai Medical Center
John M. Goldberg, MD, H3 Biomedicine
Richard Gorlick, MD, MD Anderson Cancer Center
Lee Helman, MD, University of Southern California/Children’s Hospital Los Angeles
Eugenie S. Kleinerman, MD, MD Anderson Cancer Center
Andrew S. Kraft, MD, University of Arizona Cancer Center
Marc Ladanyi, MD, Memorial Sloan-Kettering Cancer Center
David M. Loeb, MD, PhD, Children’s Hospital at Montefiore
Crystal Mackall, MD, Stanford Cancer Institute
Robert Maki, MD, PhD, Cold Spring Harbor Laboratory
Paul A. Meyers, MD, Memorial Sloan-Kettering Cancer Center
Alberto Pappo, MD, St. Jude Children’s Research Hospital
Shreyaskumar Patel, MD, MD Anderson Cancer Center
Peter Pisters, MD, University Health Network
R. Lor Randall, MD, FACS, Huntsman Cancer Center, University of Utah
Richard F. Riedel, MD, Duke University Medical Center
Scott Schuetze, MD, University of Michigan
Samuel Singer, MD, Memorial Sloan-Kettering Cancer Center
Poul HB Sorensen, MD, PhD, University of British Columbia
William D. Tap, MD, Memorial Sloan-Kettering Cancer Center
Jonathan Trent, MD, PhD, Sylvester Comprehensive Cancer Center
Matt van de Rijn, MD, Stanford School of Medicine

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The Sarcoma Journal
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Patient resources from SFA

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Helping your patient on the sarcoma journey

The Sarcoma Foundation of America offers patient resources that explain what sarcoma is, the various subtypes, how sarcomas are diagnosed, and treatment options. Information is available online at https://www.curesarcoma.org/patient-resources/

Publications
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From the Sarcoma Foundation of America
Helping your patient on the sarcoma journey
Helping your patient on the sarcoma journey

The Sarcoma Foundation of America offers patient resources that explain what sarcoma is, the various subtypes, how sarcomas are diagnosed, and treatment options. Information is available online at https://www.curesarcoma.org/patient-resources/

The Sarcoma Foundation of America offers patient resources that explain what sarcoma is, the various subtypes, how sarcomas are diagnosed, and treatment options. Information is available online at https://www.curesarcoma.org/patient-resources/

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The Sarcoma Journal
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The Sarcoma Journal
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Apply for a Grant

Article Type
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Changed
Sarcoma Foundation of America Research Grants

The Sarcoma Foundation of America has developed a program to provide grants to investigators interested in translational science sarcoma research.  Funding of up to $50,000, allowing up to 10 percent indirect costs, is available to cover equipment and supplies in support of research on the etiology, molecular biology, pathogenesis, diagnosis, and treatment of human sarcomas.

In accordance with the mission of the Foundation, research involving the development of novel agents against sarcoma, or research that could potentially lead to the development of novel agents against sarcoma, is eligible for this funding.

Please refer to SFA's Grants FAQ for additional information.

Researchers must submit proposals electronically at proposalCENTRAL.  First-time users will be required to register and complete a professional profile in order to apply for an SFA research grant.  The SFA does not accept applications via e-mail.

For more information, visit the SFA website at https://www.curesarcoma.org/research/apply-for-a-grant/

Publications
The Sarcoma Journal
MDedge Hematology and Oncology
Topics
Sarcoma & GIST
Sections
From the Sarcoma Foundation of America
Sarcoma Foundation of America Research Grants
Sarcoma Foundation of America Research Grants

The Sarcoma Foundation of America has developed a program to provide grants to investigators interested in translational science sarcoma research.  Funding of up to $50,000, allowing up to 10 percent indirect costs, is available to cover equipment and supplies in support of research on the etiology, molecular biology, pathogenesis, diagnosis, and treatment of human sarcomas.

In accordance with the mission of the Foundation, research involving the development of novel agents against sarcoma, or research that could potentially lead to the development of novel agents against sarcoma, is eligible for this funding.

Please refer to SFA's Grants FAQ for additional information.

Researchers must submit proposals electronically at proposalCENTRAL.  First-time users will be required to register and complete a professional profile in order to apply for an SFA research grant.  The SFA does not accept applications via e-mail.

For more information, visit the SFA website at https://www.curesarcoma.org/research/apply-for-a-grant/

The Sarcoma Foundation of America has developed a program to provide grants to investigators interested in translational science sarcoma research.  Funding of up to $50,000, allowing up to 10 percent indirect costs, is available to cover equipment and supplies in support of research on the etiology, molecular biology, pathogenesis, diagnosis, and treatment of human sarcomas.

In accordance with the mission of the Foundation, research involving the development of novel agents against sarcoma, or research that could potentially lead to the development of novel agents against sarcoma, is eligible for this funding.

Please refer to SFA's Grants FAQ for additional information.

Researchers must submit proposals electronically at proposalCENTRAL.  First-time users will be required to register and complete a professional profile in order to apply for an SFA research grant.  The SFA does not accept applications via e-mail.

For more information, visit the SFA website at https://www.curesarcoma.org/research/apply-for-a-grant/

Publications
The Sarcoma Journal
MDedge Hematology and Oncology
Publications
The Sarcoma Journal
MDedge Hematology and Oncology
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Sarcoma & GIST
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Article
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The Sarcoma Foundation of America: Funding research, advocating for patients

Article Type
Article
Changed

The Sarcoma Foundation of America is a national organization dedicated to increasing sarcoma awareness and research. Through its advocacy and fundraising efforts, the foundation has supported 115 sarcoma research grants since 2003, along with two large American Society of Clinical Oncology (ASCO) Foundation Clinical Research grants worth $450,000. The SFA has also funded six ASCO Young Investigator Awards and has started a large research based initiative called the Sarcoma Patient Registry, designed to increase research performed on sarcoma and to facilitate clinical trials.

To learn more about the Sarcoma Foundation of America, visit their website.

Publications
The Sarcoma Journal
MDedge Hematology and Oncology
Topics
Sarcoma & GIST
Sections
From the Sarcoma Foundation of America

The Sarcoma Foundation of America is a national organization dedicated to increasing sarcoma awareness and research. Through its advocacy and fundraising efforts, the foundation has supported 115 sarcoma research grants since 2003, along with two large American Society of Clinical Oncology (ASCO) Foundation Clinical Research grants worth $450,000. The SFA has also funded six ASCO Young Investigator Awards and has started a large research based initiative called the Sarcoma Patient Registry, designed to increase research performed on sarcoma and to facilitate clinical trials.

To learn more about the Sarcoma Foundation of America, visit their website.

The Sarcoma Foundation of America is a national organization dedicated to increasing sarcoma awareness and research. Through its advocacy and fundraising efforts, the foundation has supported 115 sarcoma research grants since 2003, along with two large American Society of Clinical Oncology (ASCO) Foundation Clinical Research grants worth $450,000. The SFA has also funded six ASCO Young Investigator Awards and has started a large research based initiative called the Sarcoma Patient Registry, designed to increase research performed on sarcoma and to facilitate clinical trials.

To learn more about the Sarcoma Foundation of America, visit their website.

Publications
The Sarcoma Journal
MDedge Hematology and Oncology
Publications
The Sarcoma Journal
MDedge Hematology and Oncology
Topics
Sarcoma & GIST
Article Type
Article
Sections
From the Sarcoma Foundation of America
Disallow All Ads
Content Gating
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Default
Gate On Date
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Absurdity

Article Type
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Changed
Author(s)
Alan Rockoff, MD

Absurdity is everywhere you look. Or don’t look.

As the old comedian Henny Youngman might have said:

Take my prior authorizations. Please!
 

Prior authorizations

1. Marissa had been taking isotretinoin for 2 months. She learned that three 20-mg capsules would cost her less than the two 30-mg capsules she’d been on.

cglade/E+/Getty Images
Her insurer wanted Prior Authorization. I filled out their form.

Denied: “You have not provided a valid medical reason for prescribing more than two pills per day.”

My secretary called them. The insurance representative (Pharmacist? Clerk? Gal who stopped by to read the gas meter?) couldn’t help. “When I input your information, it issues a denial.” (Who is “it”? Watson’s evil twin Jensen?)

 

 


I got on the phone.

“Forgive me,” I said, gently, “but Marissa has been taking isotretinoin for 2 months, 60-mg per day. She was taking two 30’s. I want her to have three 20’s. They both add up to 60 mg. It’s the same dose. Why do you need to authorize it again?”

“Let me input that,” she replied. “Oh, now it’s accepting it. Your patient can have up to four pills a day.”


I was going to say she only needs three, but kept my mouth shut. Maybe Jensen only authorizes even numbers. 2. Danny has the worst atopic dermatitis I’ve ever seen. It’s all over him and never lets up. Topical steroids don’t touch it. Even prednisone – he’s had plenty over the years – barely makes a dent. I put in a Prior Authorization request for dupilumab.

 

 


“Your request for dupilumab has been denied,” read the insurer’s reply. “You have not shown failure with tacrolimus ointment or crisaborole.”

Say what? Prednisone didn’t help, and they expect tacrolimus or crisaborole to do the job?

I prescribed tacrolimus (which doesn’t come in a big enough tube to cover Daniel’s affected area anyway). It failed. Amazing.


“Your request for dupilumab has been denied,” said the insurer. “You have not also shown failure with crisaborole.”

 

 


Really? OK, I prescribed crisaborole. They denied coverage for it.

Now I was really getting into this. I wrote them. “I prescribed crisaborole.” I observed, “because you asked me to.”

They approved crisaborole. It failed. I reapplied for dupilumab. No response.

I called the insurer’s medical director, on a mobile with a Missouri exchange. After some telephone tag, he called back. “I cannot discuss this case with you,” he said, “because I have already made my determination.” Then he hung up without telling me what his determination was.

 

 


Further phone calls went unanswered. I thought Missouri was the Show-Me state.

The patient remained miserable. I decided to try one last time and wrote a long, sarcastic letter, detailing the whole episode. My secretary sent it off.

They approved dupilumab within the hour.


Malice? Nah, that’s giving them too much credit.

 

 


Now all Daniel has to do is improve.

Patient privacy!

Some news from abroad: what we know as HIPAA is called the “Data Protection Act” in the United Kingdom.

Dr. Alan Rockoff
In Jewish synagogues, the rabbi may offer prayers for members of the community who are ill. Or at least rabbis used to:


“We are obliged to conform to the demands of the Data Protection Act, and this specifically applies to the rabbi publicly mentioning the names of individuals who are unwell. The rabbi can only mention specific individuals with their permission or that of a relative designated by the sick person to do so. Anyone wishing for the rabbi to say a public prayer on their behalf must contact him directly by phone, text, or e-mail. To do anything else is breaking the law.”

 

 


If someone breaks this law, perhaps the rabbi can assist with atonement.

In any event, henceforth all entreaties to the Almighty must be encrypted. At least in the U.K.

What???!!!

Marina showed me her sunscreen. The label read, “Protects against UVA and UVB rays.”

“What’s the problem?” I asked.

 

 


She showed me our American Academy of Dermatology-produced sunscreen handout, which recommends “a broad-spectrum sunscreen that protects against both UVA and UVB rays, both of which cause cancer.”

“Does this mean my sunscreen causes cancer?” asked Marina.

“Not to worry,” I assured her.

I sighed and wafted a small prayer heavenward. Encrypted, of course.

Lo, the answer from above may tarry, but He will never forget His password.

Dr. Rockoff practices dermatology in Brookline, Mass., and is a longtime contributor to Dermatology News. He serves on the clinical faculty at Tufts University, Boston, and has taught senior medical students and other trainees for 30 years. His second book, “Act Like a Doctor, Think Like a Patient,” is available at amazon.com and barnesandnoble.com. Write to him at [email protected].

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Absurdity is everywhere you look. Or don’t look.

As the old comedian Henny Youngman might have said:

Take my prior authorizations. Please!
 

Prior authorizations

1. Marissa had been taking isotretinoin for 2 months. She learned that three 20-mg capsules would cost her less than the two 30-mg capsules she’d been on.

cglade/E+/Getty Images
Her insurer wanted Prior Authorization. I filled out their form.

Denied: “You have not provided a valid medical reason for prescribing more than two pills per day.”

My secretary called them. The insurance representative (Pharmacist? Clerk? Gal who stopped by to read the gas meter?) couldn’t help. “When I input your information, it issues a denial.” (Who is “it”? Watson’s evil twin Jensen?)

 

 


I got on the phone.

“Forgive me,” I said, gently, “but Marissa has been taking isotretinoin for 2 months, 60-mg per day. She was taking two 30’s. I want her to have three 20’s. They both add up to 60 mg. It’s the same dose. Why do you need to authorize it again?”

“Let me input that,” she replied. “Oh, now it’s accepting it. Your patient can have up to four pills a day.”


I was going to say she only needs three, but kept my mouth shut. Maybe Jensen only authorizes even numbers. 2. Danny has the worst atopic dermatitis I’ve ever seen. It’s all over him and never lets up. Topical steroids don’t touch it. Even prednisone – he’s had plenty over the years – barely makes a dent. I put in a Prior Authorization request for dupilumab.

 

 


“Your request for dupilumab has been denied,” read the insurer’s reply. “You have not shown failure with tacrolimus ointment or crisaborole.”

Say what? Prednisone didn’t help, and they expect tacrolimus or crisaborole to do the job?

I prescribed tacrolimus (which doesn’t come in a big enough tube to cover Daniel’s affected area anyway). It failed. Amazing.


“Your request for dupilumab has been denied,” said the insurer. “You have not also shown failure with crisaborole.”

 

 


Really? OK, I prescribed crisaborole. They denied coverage for it.

Now I was really getting into this. I wrote them. “I prescribed crisaborole.” I observed, “because you asked me to.”

They approved crisaborole. It failed. I reapplied for dupilumab. No response.

I called the insurer’s medical director, on a mobile with a Missouri exchange. After some telephone tag, he called back. “I cannot discuss this case with you,” he said, “because I have already made my determination.” Then he hung up without telling me what his determination was.

 

 


Further phone calls went unanswered. I thought Missouri was the Show-Me state.

The patient remained miserable. I decided to try one last time and wrote a long, sarcastic letter, detailing the whole episode. My secretary sent it off.

They approved dupilumab within the hour.


Malice? Nah, that’s giving them too much credit.

 

 


Now all Daniel has to do is improve.

Patient privacy!

Some news from abroad: what we know as HIPAA is called the “Data Protection Act” in the United Kingdom.

Dr. Alan Rockoff
In Jewish synagogues, the rabbi may offer prayers for members of the community who are ill. Or at least rabbis used to:


“We are obliged to conform to the demands of the Data Protection Act, and this specifically applies to the rabbi publicly mentioning the names of individuals who are unwell. The rabbi can only mention specific individuals with their permission or that of a relative designated by the sick person to do so. Anyone wishing for the rabbi to say a public prayer on their behalf must contact him directly by phone, text, or e-mail. To do anything else is breaking the law.”

 

 


If someone breaks this law, perhaps the rabbi can assist with atonement.

In any event, henceforth all entreaties to the Almighty must be encrypted. At least in the U.K.

What???!!!

Marina showed me her sunscreen. The label read, “Protects against UVA and UVB rays.”

“What’s the problem?” I asked.

 

 


She showed me our American Academy of Dermatology-produced sunscreen handout, which recommends “a broad-spectrum sunscreen that protects against both UVA and UVB rays, both of which cause cancer.”

“Does this mean my sunscreen causes cancer?” asked Marina.

“Not to worry,” I assured her.

I sighed and wafted a small prayer heavenward. Encrypted, of course.

Lo, the answer from above may tarry, but He will never forget His password.

Dr. Rockoff practices dermatology in Brookline, Mass., and is a longtime contributor to Dermatology News. He serves on the clinical faculty at Tufts University, Boston, and has taught senior medical students and other trainees for 30 years. His second book, “Act Like a Doctor, Think Like a Patient,” is available at amazon.com and barnesandnoble.com. Write to him at [email protected].

Absurdity is everywhere you look. Or don’t look.

As the old comedian Henny Youngman might have said:

Take my prior authorizations. Please!
 

Prior authorizations

1. Marissa had been taking isotretinoin for 2 months. She learned that three 20-mg capsules would cost her less than the two 30-mg capsules she’d been on.

cglade/E+/Getty Images
Her insurer wanted Prior Authorization. I filled out their form.

Denied: “You have not provided a valid medical reason for prescribing more than two pills per day.”

My secretary called them. The insurance representative (Pharmacist? Clerk? Gal who stopped by to read the gas meter?) couldn’t help. “When I input your information, it issues a denial.” (Who is “it”? Watson’s evil twin Jensen?)

 

 


I got on the phone.

“Forgive me,” I said, gently, “but Marissa has been taking isotretinoin for 2 months, 60-mg per day. She was taking two 30’s. I want her to have three 20’s. They both add up to 60 mg. It’s the same dose. Why do you need to authorize it again?”

“Let me input that,” she replied. “Oh, now it’s accepting it. Your patient can have up to four pills a day.”


I was going to say she only needs three, but kept my mouth shut. Maybe Jensen only authorizes even numbers. 2. Danny has the worst atopic dermatitis I’ve ever seen. It’s all over him and never lets up. Topical steroids don’t touch it. Even prednisone – he’s had plenty over the years – barely makes a dent. I put in a Prior Authorization request for dupilumab.

 

 


“Your request for dupilumab has been denied,” read the insurer’s reply. “You have not shown failure with tacrolimus ointment or crisaborole.”

Say what? Prednisone didn’t help, and they expect tacrolimus or crisaborole to do the job?

I prescribed tacrolimus (which doesn’t come in a big enough tube to cover Daniel’s affected area anyway). It failed. Amazing.


“Your request for dupilumab has been denied,” said the insurer. “You have not also shown failure with crisaborole.”

 

 


Really? OK, I prescribed crisaborole. They denied coverage for it.

Now I was really getting into this. I wrote them. “I prescribed crisaborole.” I observed, “because you asked me to.”

They approved crisaborole. It failed. I reapplied for dupilumab. No response.

I called the insurer’s medical director, on a mobile with a Missouri exchange. After some telephone tag, he called back. “I cannot discuss this case with you,” he said, “because I have already made my determination.” Then he hung up without telling me what his determination was.

 

 


Further phone calls went unanswered. I thought Missouri was the Show-Me state.

The patient remained miserable. I decided to try one last time and wrote a long, sarcastic letter, detailing the whole episode. My secretary sent it off.

They approved dupilumab within the hour.


Malice? Nah, that’s giving them too much credit.

 

 


Now all Daniel has to do is improve.

Patient privacy!

Some news from abroad: what we know as HIPAA is called the “Data Protection Act” in the United Kingdom.

Dr. Alan Rockoff
In Jewish synagogues, the rabbi may offer prayers for members of the community who are ill. Or at least rabbis used to:


“We are obliged to conform to the demands of the Data Protection Act, and this specifically applies to the rabbi publicly mentioning the names of individuals who are unwell. The rabbi can only mention specific individuals with their permission or that of a relative designated by the sick person to do so. Anyone wishing for the rabbi to say a public prayer on their behalf must contact him directly by phone, text, or e-mail. To do anything else is breaking the law.”

 

 


If someone breaks this law, perhaps the rabbi can assist with atonement.

In any event, henceforth all entreaties to the Almighty must be encrypted. At least in the U.K.

What???!!!

Marina showed me her sunscreen. The label read, “Protects against UVA and UVB rays.”

“What’s the problem?” I asked.

 

 


She showed me our American Academy of Dermatology-produced sunscreen handout, which recommends “a broad-spectrum sunscreen that protects against both UVA and UVB rays, both of which cause cancer.”

“Does this mean my sunscreen causes cancer?” asked Marina.

“Not to worry,” I assured her.

I sighed and wafted a small prayer heavenward. Encrypted, of course.

Lo, the answer from above may tarry, but He will never forget His password.

Dr. Rockoff practices dermatology in Brookline, Mass., and is a longtime contributor to Dermatology News. He serves on the clinical faculty at Tufts University, Boston, and has taught senior medical students and other trainees for 30 years. His second book, “Act Like a Doctor, Think Like a Patient,” is available at amazon.com and barnesandnoble.com. Write to him at [email protected].

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ALUR: Alectinib topped chemo in pretreated ALK-positive NSCLC

Cross-trial comparisons are revealing
Article Type
News
Changed
Author(s)
Amy Karon

 

The second-generation anaplastic lymphoma kinase (ALK) inhibitor alectinib (Alcensa) topped chemotherapy in crizotinib-pretreated ALK+ non–small cell lung cancer (NSCLC), according to results from the phase 3 ALUR trial.

Median investigator-assessed progression-free survival was 9.6 months with alectinib and 1.4 months with chemotherapy (hazard ratio, 0.15; P less than .001), reported Silvia Novello, MD, PhD, of University of Turin (Italy) and her associates. Among patients with measurable central nervous system disease, the rate of CNS objective response was significantly higher for alectinib (54%) versus chemotherapy (0%; P less than .001), Dr. Novello and her associates reported in Annals of Oncology.

The multicenter, open-label ALUR trial was the first to directly compare alectinib with standard chemotherapy in patients with ALK-rearranged NSCLC that previously had been treated with both platinum-based chemotherapy and crizotinib. In all, 107 patients were randomly assigned on a 2:1 basis to receive either alectinib (600 mg twice daily) or chemotherapy (clinician’s choice of pemetrexed 500 mg/m2 or docetaxel 75 mg/m2 every 3 weeks).

A blinded independent review committee calculated median progression-free survival (PFS) times that were 2.5 months shorter for alectinib and 0.2 months longer for chemotherapy. Consequently, the hazard ratio for PFS was somewhat attenuated at 0.32 but remained highly significant (95% confidence interval, 0.17-0.59). “[Median] PFS with alectinib in ALUR has exceeded that observed with [second-line] ceritinib” during the ASCEND-5 study, the researchers wrote. In ASCEND-5, median independent review committee–assessed PFS time was 5.4 months, which is 1.7 months shorter than that for alectinib in ALUR. In each study, chemotherapy yielded a median PFS time of 1.6 months, which facilitated intertrial comparisons, they wrote.

Rates of all-grade and serious adverse events were similar between arms in ALUR. Alectinib therapy caused no fatal adverse events, while chemotherapy was associated with one fatality deemed unrelated to treatment. Alectinib was more likely to produce constipation, dyspnea, and hyperbilirubinemia, while chemotherapy was more likely to cause nausea, alopecia, neutropenia, diarrhea, pruritus, stomatitis, and bacterial pneumonia. Although patients stayed on alectinib a median of 14 weeks longer than on chemotherapy, they were less likely to stop alectinib (6%) than chemotherapy (9%) for adverse events.

Dr. Novello disclosed personal fees from Roche, which markets alectinib. Eleven coinvestigators also disclosed employment, stock ownership, or other financial ties to Roche.

SOURCE: Novello S et al. Ann Oncol. 2018 Apr 14. doi: 10.1093/annonc/mdy121.

Body

 

Questions about which next-generation anaplastic lymphoma kinase (ALK) tyrosine kinase inhibitor is best for treatment-naive ALK+ NSCLC will incite “vigorous debate and the inevitable cross-trial comparison that everyone frowns upon but does anyway,” wrote Misako Nagasaka, MD; Viola W. Zhu, MD, PhD; and Sai-Hong Ignatius Ou, MD, PhD, in an editorial accompanying the study in Annals of Oncology.

Median progression-free survival time in ALUR was 7.1 months, versus 5.4 months in the similarly designed ASCEND-5 trial of second-line ceritinib in ALK+ NSCLC, they noted. “ALUR seems to confirm the superiority of alectinib [over ceritinib] in the post-crizotinib setting.”

Similarly, first-line alectinib produced a longer median progression-free survival time (25.7 months) in the ALEX trial than did first-line ceritinib (16.6 months) in the ASCEND-4 trial, the editorialists noted.

They called brigatinib “the one ALK TKI [anaplastic lymphoma kinase tyrosine kinase inhibitor] that can challenge alectinib.” The global phase 3 Brigatinib 3001 trial will directly compare brigatinib with alectinib in the post-chemotherapy and post-crizotinib setting.
 

Dr. Nagasaka is with Wayne State University, Detroit; she reported having no conflicts of interest. Dr. Zhu and Dr. Ou are with the University of California, Irvine; they disclosed ties to Roche/Genentech, Pfizer, and Takeda/Ariad. These comments summarize their editorial (Ann Oncol. 2018 Apr 14. doi: 10.1093/annonc/mdy144 )

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Body

 

Questions about which next-generation anaplastic lymphoma kinase (ALK) tyrosine kinase inhibitor is best for treatment-naive ALK+ NSCLC will incite “vigorous debate and the inevitable cross-trial comparison that everyone frowns upon but does anyway,” wrote Misako Nagasaka, MD; Viola W. Zhu, MD, PhD; and Sai-Hong Ignatius Ou, MD, PhD, in an editorial accompanying the study in Annals of Oncology.

Median progression-free survival time in ALUR was 7.1 months, versus 5.4 months in the similarly designed ASCEND-5 trial of second-line ceritinib in ALK+ NSCLC, they noted. “ALUR seems to confirm the superiority of alectinib [over ceritinib] in the post-crizotinib setting.”

Similarly, first-line alectinib produced a longer median progression-free survival time (25.7 months) in the ALEX trial than did first-line ceritinib (16.6 months) in the ASCEND-4 trial, the editorialists noted.

They called brigatinib “the one ALK TKI [anaplastic lymphoma kinase tyrosine kinase inhibitor] that can challenge alectinib.” The global phase 3 Brigatinib 3001 trial will directly compare brigatinib with alectinib in the post-chemotherapy and post-crizotinib setting.
 

Dr. Nagasaka is with Wayne State University, Detroit; she reported having no conflicts of interest. Dr. Zhu and Dr. Ou are with the University of California, Irvine; they disclosed ties to Roche/Genentech, Pfizer, and Takeda/Ariad. These comments summarize their editorial (Ann Oncol. 2018 Apr 14. doi: 10.1093/annonc/mdy144 )

Body

 

Questions about which next-generation anaplastic lymphoma kinase (ALK) tyrosine kinase inhibitor is best for treatment-naive ALK+ NSCLC will incite “vigorous debate and the inevitable cross-trial comparison that everyone frowns upon but does anyway,” wrote Misako Nagasaka, MD; Viola W. Zhu, MD, PhD; and Sai-Hong Ignatius Ou, MD, PhD, in an editorial accompanying the study in Annals of Oncology.

Median progression-free survival time in ALUR was 7.1 months, versus 5.4 months in the similarly designed ASCEND-5 trial of second-line ceritinib in ALK+ NSCLC, they noted. “ALUR seems to confirm the superiority of alectinib [over ceritinib] in the post-crizotinib setting.”

Similarly, first-line alectinib produced a longer median progression-free survival time (25.7 months) in the ALEX trial than did first-line ceritinib (16.6 months) in the ASCEND-4 trial, the editorialists noted.

They called brigatinib “the one ALK TKI [anaplastic lymphoma kinase tyrosine kinase inhibitor] that can challenge alectinib.” The global phase 3 Brigatinib 3001 trial will directly compare brigatinib with alectinib in the post-chemotherapy and post-crizotinib setting.
 

Dr. Nagasaka is with Wayne State University, Detroit; she reported having no conflicts of interest. Dr. Zhu and Dr. Ou are with the University of California, Irvine; they disclosed ties to Roche/Genentech, Pfizer, and Takeda/Ariad. These comments summarize their editorial (Ann Oncol. 2018 Apr 14. doi: 10.1093/annonc/mdy144 )

Title
Cross-trial comparisons are revealing
Cross-trial comparisons are revealing

 

The second-generation anaplastic lymphoma kinase (ALK) inhibitor alectinib (Alcensa) topped chemotherapy in crizotinib-pretreated ALK+ non–small cell lung cancer (NSCLC), according to results from the phase 3 ALUR trial.

Median investigator-assessed progression-free survival was 9.6 months with alectinib and 1.4 months with chemotherapy (hazard ratio, 0.15; P less than .001), reported Silvia Novello, MD, PhD, of University of Turin (Italy) and her associates. Among patients with measurable central nervous system disease, the rate of CNS objective response was significantly higher for alectinib (54%) versus chemotherapy (0%; P less than .001), Dr. Novello and her associates reported in Annals of Oncology.

The multicenter, open-label ALUR trial was the first to directly compare alectinib with standard chemotherapy in patients with ALK-rearranged NSCLC that previously had been treated with both platinum-based chemotherapy and crizotinib. In all, 107 patients were randomly assigned on a 2:1 basis to receive either alectinib (600 mg twice daily) or chemotherapy (clinician’s choice of pemetrexed 500 mg/m2 or docetaxel 75 mg/m2 every 3 weeks).

A blinded independent review committee calculated median progression-free survival (PFS) times that were 2.5 months shorter for alectinib and 0.2 months longer for chemotherapy. Consequently, the hazard ratio for PFS was somewhat attenuated at 0.32 but remained highly significant (95% confidence interval, 0.17-0.59). “[Median] PFS with alectinib in ALUR has exceeded that observed with [second-line] ceritinib” during the ASCEND-5 study, the researchers wrote. In ASCEND-5, median independent review committee–assessed PFS time was 5.4 months, which is 1.7 months shorter than that for alectinib in ALUR. In each study, chemotherapy yielded a median PFS time of 1.6 months, which facilitated intertrial comparisons, they wrote.

Rates of all-grade and serious adverse events were similar between arms in ALUR. Alectinib therapy caused no fatal adverse events, while chemotherapy was associated with one fatality deemed unrelated to treatment. Alectinib was more likely to produce constipation, dyspnea, and hyperbilirubinemia, while chemotherapy was more likely to cause nausea, alopecia, neutropenia, diarrhea, pruritus, stomatitis, and bacterial pneumonia. Although patients stayed on alectinib a median of 14 weeks longer than on chemotherapy, they were less likely to stop alectinib (6%) than chemotherapy (9%) for adverse events.

Dr. Novello disclosed personal fees from Roche, which markets alectinib. Eleven coinvestigators also disclosed employment, stock ownership, or other financial ties to Roche.

SOURCE: Novello S et al. Ann Oncol. 2018 Apr 14. doi: 10.1093/annonc/mdy121.

 

The second-generation anaplastic lymphoma kinase (ALK) inhibitor alectinib (Alcensa) topped chemotherapy in crizotinib-pretreated ALK+ non–small cell lung cancer (NSCLC), according to results from the phase 3 ALUR trial.

Median investigator-assessed progression-free survival was 9.6 months with alectinib and 1.4 months with chemotherapy (hazard ratio, 0.15; P less than .001), reported Silvia Novello, MD, PhD, of University of Turin (Italy) and her associates. Among patients with measurable central nervous system disease, the rate of CNS objective response was significantly higher for alectinib (54%) versus chemotherapy (0%; P less than .001), Dr. Novello and her associates reported in Annals of Oncology.

The multicenter, open-label ALUR trial was the first to directly compare alectinib with standard chemotherapy in patients with ALK-rearranged NSCLC that previously had been treated with both platinum-based chemotherapy and crizotinib. In all, 107 patients were randomly assigned on a 2:1 basis to receive either alectinib (600 mg twice daily) or chemotherapy (clinician’s choice of pemetrexed 500 mg/m2 or docetaxel 75 mg/m2 every 3 weeks).

A blinded independent review committee calculated median progression-free survival (PFS) times that were 2.5 months shorter for alectinib and 0.2 months longer for chemotherapy. Consequently, the hazard ratio for PFS was somewhat attenuated at 0.32 but remained highly significant (95% confidence interval, 0.17-0.59). “[Median] PFS with alectinib in ALUR has exceeded that observed with [second-line] ceritinib” during the ASCEND-5 study, the researchers wrote. In ASCEND-5, median independent review committee–assessed PFS time was 5.4 months, which is 1.7 months shorter than that for alectinib in ALUR. In each study, chemotherapy yielded a median PFS time of 1.6 months, which facilitated intertrial comparisons, they wrote.

Rates of all-grade and serious adverse events were similar between arms in ALUR. Alectinib therapy caused no fatal adverse events, while chemotherapy was associated with one fatality deemed unrelated to treatment. Alectinib was more likely to produce constipation, dyspnea, and hyperbilirubinemia, while chemotherapy was more likely to cause nausea, alopecia, neutropenia, diarrhea, pruritus, stomatitis, and bacterial pneumonia. Although patients stayed on alectinib a median of 14 weeks longer than on chemotherapy, they were less likely to stop alectinib (6%) than chemotherapy (9%) for adverse events.

Dr. Novello disclosed personal fees from Roche, which markets alectinib. Eleven coinvestigators also disclosed employment, stock ownership, or other financial ties to Roche.

SOURCE: Novello S et al. Ann Oncol. 2018 Apr 14. doi: 10.1093/annonc/mdy121.

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Key clinical point: Alectinib topped chemotherapy in patients with advanced/metastatic crizotinib-pretreated, ALK-positive NSCLC.

Major finding: Median investigator-assessed progression-free survival was 9.6 months with alectinib and 1.4 months with chemotherapy (hazard ratio, 0.15; P less than .001).

Study details: ALUR, which is a randomized, multicenter, open-label, phase 3 trial of 107 patients.

Disclosures: Dr. Novello disclosed personal fees from Roche, which markets alectinib. Eleven coinvestigators also disclosed employment, stock ownership, or other financial ties to Roche.

Source: Novello S et al. Ann Oncol. 2018 Apr 14. doi: 10.1093/annonc/mdy121

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Suicidality assessment of people with autism needs better tools

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Author(s)
Mitchel L. Zoler, PhD

 

WASHINGTON – People with autism spectrum disorder face a double whammy on suicide risk: They have cognitive, social, and emotional behaviors that increase their vulnerability to suicide, but they also often find it difficult to communicate their depression and suicidality and so may often go unrecognized as suicidal. Or if they are identified, conventional prevention interventions might be less effective.

To try to address this, clinicians are trying to develop a suicide screening questionnaire that is better geared for use on people with autism spectrum disorder (ASD), Jacqueline Wynn, PhD said at the annual conference of the American Association of Suicidology.

Mitchel L. Zoler/MDedge News
Dr. Jacqueline Wynn
“We’re gathering data to adapt the Ask Suicide-Screening Questions [ASQ] to add specific ASD-specific questions,” explained Dr. Wynn, a psychologist and director of the Center of Autism Spectrum Disorders at Nationwide Children’s Hospital in Columbus, Ohio. “We’re collecting data at Nationwide Children and trying to involve other centers” in a project sponsored by the National Institute of Mental Health that Dr. Wynn estimated might take at least 2 more years to complete and come up with a validated, revised ASQ that’s better suited to children, adolescents, and adults with ASD.

Many people with ASD “have impaired language capabilities” that make their expressions of depression and suicide ideation more complex, she observed.



“The point is that we need better measures in the ASQ,” said John P. Ackerman, PhD, a clinical psychologist and suicide prevention coordinator at the Center for Suicide Prevention and Research at Nationwide Children’s. “There is a misperception that because people with autism don’t express their emotions and can’t always access the words they don’t have suicide ideation. They do,” he declared in an interview.

People with ASD have high rates of depression and anxiety, decreased inhibitory control and emotional regulation, rigidity or thought, and difficulty asking for help or accepting help. Youth with ASD undergo psychiatric hospitalization more than 10-fold more often than similarly aged youth without a psychiatric diagnosis, Dr. Ackerman noted. In one recent study of 374 adults with Asperger’s syndrome, two-thirds reported having suicidal ideation and one-third self-reported a planned or attempted suicide (Lancet Psychiatry. 2014 Jul;1[2]:142-7).

The risks that people with ASD have for depression and suicide contrasts with the way clinicians currently address this issue. “There are many gaps” in suicide-risk assessment and prevention interventions aimed at people with ASD, he said. For example, a depression symptom checklist that asks whether someone is withdrawn or feeling disconnected focuses on commonplace characteristics among people with ASD.

 

 


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Dr. John P. Ackerman
Dr. Ackerman, Dr. Wynn, and their associates at Nationwide Children’s already have applied these understandings to change the way they deal with people with ASD. “We make sure that the staff dealing with people with autism know how to respond,” Dr. Ackerman said. But the new ASQ under development represents a potentially important step forward. “It’s embarrassing to say that we don’t have the necessary tools” to deal effectively with depression and suicidality in people with ASD, he admitted.

The recognition that people with ASD need tailored methods for both identifying and intervening with suicidality appears to be part of an emerging appreciation by clinicians who work on suicide prevention of the “need to meet people where they are,” Dr. Ackerman said. Similar approaches might be needed for various ethnic and racial groups, gays, transgender people, those who are hearing impaired, and others who might respond better to novel approaches.

Dr. Wynn and Dr. Ackerman had no disclosures.
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WASHINGTON – People with autism spectrum disorder face a double whammy on suicide risk: They have cognitive, social, and emotional behaviors that increase their vulnerability to suicide, but they also often find it difficult to communicate their depression and suicidality and so may often go unrecognized as suicidal. Or if they are identified, conventional prevention interventions might be less effective.

To try to address this, clinicians are trying to develop a suicide screening questionnaire that is better geared for use on people with autism spectrum disorder (ASD), Jacqueline Wynn, PhD said at the annual conference of the American Association of Suicidology.

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Dr. Jacqueline Wynn
“We’re gathering data to adapt the Ask Suicide-Screening Questions [ASQ] to add specific ASD-specific questions,” explained Dr. Wynn, a psychologist and director of the Center of Autism Spectrum Disorders at Nationwide Children’s Hospital in Columbus, Ohio. “We’re collecting data at Nationwide Children and trying to involve other centers” in a project sponsored by the National Institute of Mental Health that Dr. Wynn estimated might take at least 2 more years to complete and come up with a validated, revised ASQ that’s better suited to children, adolescents, and adults with ASD.

Many people with ASD “have impaired language capabilities” that make their expressions of depression and suicide ideation more complex, she observed.



“The point is that we need better measures in the ASQ,” said John P. Ackerman, PhD, a clinical psychologist and suicide prevention coordinator at the Center for Suicide Prevention and Research at Nationwide Children’s. “There is a misperception that because people with autism don’t express their emotions and can’t always access the words they don’t have suicide ideation. They do,” he declared in an interview.

People with ASD have high rates of depression and anxiety, decreased inhibitory control and emotional regulation, rigidity or thought, and difficulty asking for help or accepting help. Youth with ASD undergo psychiatric hospitalization more than 10-fold more often than similarly aged youth without a psychiatric diagnosis, Dr. Ackerman noted. In one recent study of 374 adults with Asperger’s syndrome, two-thirds reported having suicidal ideation and one-third self-reported a planned or attempted suicide (Lancet Psychiatry. 2014 Jul;1[2]:142-7).

The risks that people with ASD have for depression and suicide contrasts with the way clinicians currently address this issue. “There are many gaps” in suicide-risk assessment and prevention interventions aimed at people with ASD, he said. For example, a depression symptom checklist that asks whether someone is withdrawn or feeling disconnected focuses on commonplace characteristics among people with ASD.

 

 


Mitchel L. Zoler/MDedge News
Dr. John P. Ackerman
Dr. Ackerman, Dr. Wynn, and their associates at Nationwide Children’s already have applied these understandings to change the way they deal with people with ASD. “We make sure that the staff dealing with people with autism know how to respond,” Dr. Ackerman said. But the new ASQ under development represents a potentially important step forward. “It’s embarrassing to say that we don’t have the necessary tools” to deal effectively with depression and suicidality in people with ASD, he admitted.

The recognition that people with ASD need tailored methods for both identifying and intervening with suicidality appears to be part of an emerging appreciation by clinicians who work on suicide prevention of the “need to meet people where they are,” Dr. Ackerman said. Similar approaches might be needed for various ethnic and racial groups, gays, transgender people, those who are hearing impaired, and others who might respond better to novel approaches.

Dr. Wynn and Dr. Ackerman had no disclosures.

 

WASHINGTON – People with autism spectrum disorder face a double whammy on suicide risk: They have cognitive, social, and emotional behaviors that increase their vulnerability to suicide, but they also often find it difficult to communicate their depression and suicidality and so may often go unrecognized as suicidal. Or if they are identified, conventional prevention interventions might be less effective.

To try to address this, clinicians are trying to develop a suicide screening questionnaire that is better geared for use on people with autism spectrum disorder (ASD), Jacqueline Wynn, PhD said at the annual conference of the American Association of Suicidology.

Mitchel L. Zoler/MDedge News
Dr. Jacqueline Wynn
“We’re gathering data to adapt the Ask Suicide-Screening Questions [ASQ] to add specific ASD-specific questions,” explained Dr. Wynn, a psychologist and director of the Center of Autism Spectrum Disorders at Nationwide Children’s Hospital in Columbus, Ohio. “We’re collecting data at Nationwide Children and trying to involve other centers” in a project sponsored by the National Institute of Mental Health that Dr. Wynn estimated might take at least 2 more years to complete and come up with a validated, revised ASQ that’s better suited to children, adolescents, and adults with ASD.

Many people with ASD “have impaired language capabilities” that make their expressions of depression and suicide ideation more complex, she observed.



“The point is that we need better measures in the ASQ,” said John P. Ackerman, PhD, a clinical psychologist and suicide prevention coordinator at the Center for Suicide Prevention and Research at Nationwide Children’s. “There is a misperception that because people with autism don’t express their emotions and can’t always access the words they don’t have suicide ideation. They do,” he declared in an interview.

People with ASD have high rates of depression and anxiety, decreased inhibitory control and emotional regulation, rigidity or thought, and difficulty asking for help or accepting help. Youth with ASD undergo psychiatric hospitalization more than 10-fold more often than similarly aged youth without a psychiatric diagnosis, Dr. Ackerman noted. In one recent study of 374 adults with Asperger’s syndrome, two-thirds reported having suicidal ideation and one-third self-reported a planned or attempted suicide (Lancet Psychiatry. 2014 Jul;1[2]:142-7).

The risks that people with ASD have for depression and suicide contrasts with the way clinicians currently address this issue. “There are many gaps” in suicide-risk assessment and prevention interventions aimed at people with ASD, he said. For example, a depression symptom checklist that asks whether someone is withdrawn or feeling disconnected focuses on commonplace characteristics among people with ASD.

 

 


Mitchel L. Zoler/MDedge News
Dr. John P. Ackerman
Dr. Ackerman, Dr. Wynn, and their associates at Nationwide Children’s already have applied these understandings to change the way they deal with people with ASD. “We make sure that the staff dealing with people with autism know how to respond,” Dr. Ackerman said. But the new ASQ under development represents a potentially important step forward. “It’s embarrassing to say that we don’t have the necessary tools” to deal effectively with depression and suicidality in people with ASD, he admitted.

The recognition that people with ASD need tailored methods for both identifying and intervening with suicidality appears to be part of an emerging appreciation by clinicians who work on suicide prevention of the “need to meet people where they are,” Dr. Ackerman said. Similar approaches might be needed for various ethnic and racial groups, gays, transgender people, those who are hearing impaired, and others who might respond better to novel approaches.

Dr. Wynn and Dr. Ackerman had no disclosures.
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CMS floats Medicare direct provider contracting

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Gregory Twachtman

 

The Centers for Medicare & Medicaid Services wants physician input on developing direct provider contracting models to cover Medicare beneficiaries.

Under a direct provider contracting (DPC) arrangement, Medicare could pay physicians or physician groups a monthly fee to deliver a specific set of services to beneficiaries, who would gain greater access to the physicians. The physicians would be accountable for those Medicare patients’ costs and care quality.

zimmytws/gettyimages

CMS is looking at how to incorporate this concept into the Medicare ranks. On April 23, CMS issued a request for information (RFI) seeking input across a wide range of topics, including provider/state participation, beneficiary participation, payment, general model design, program integrity and beneficiary protection, and how such models would fit within the existing accountable care organization framework.

The RFI offered one possible vision on how a direct provider contracting model could work.

“Under a primary care–focused DPC model, CMS could enter into arrangements with primary care practices under which CMS would pay these participating practices a fixed per beneficiary per month (PBPM) payment to cover the primary care services the practice would be expected to furnish under the model, which may include office visits, certain office-based procedures, and other non–visit-based services covered under the physician fee schedule, and flexibility in how otherwise billable services are delivered,” the RFI states.

Physicians could also earn performance bonuses, depending on how the DPC is structured, through “performance-based incentives for total cost of care and quality.”

CMS noted it also “could test ways to reduce administrative burden though innovative changes to claims submission processes for services included in the PBPM payment under these models.”

 

 


The direct provider contracting idea grew out of a previous RFI issued in 2017 by CMS’s Center for Medicare and Medicaid Innovation to collect ideas on new ways to deliver patient-centered care. The agency released the more than 1,000 comments received from that request on the same day it issued the RFI on direct provider contracting.

In those comments, a number of physician groups offered support for a direct-contracting approach.

For example, the American Academy of Family Physicians wrote that it “sees continued growth and interest in family physicians adopting this practice model in all settings types, including rural and underserved communities.” And the AAFP suggested that the innovation center should work with DPC organizations to learn more about them.

The American College of Physicians reiterated its previous position that it “supports physician and patient choice of practice and delivery models that are accessible, ethical, and viable and that strengthen the patient-physician relationship.” But the ACP raised a number of issues that could impede access to care or result in lower quality care.

 

 


The American Medical Association offered support for “testing of models in which physicians have the ability to deliver more or different services to patients who need them and to be paid more for doing so.”

The AMA suggested that some of the models to be tested include allowing patients to contract directly with physicians, with Medicare paying its fee schedule rates and patients paying the difference; allowing patients to receive their care from DPC practices and get reimbursed by Medicare; or allowing “physicians to define a team of providers who will provide all of the treatment needed for an acute condition or management of a chronic condition, and then allowing patients who select the team to receive all of the services related to their condition from the team in return for a single predefined cost-sharing amount.”

Comments on the RFI are due May 25.

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The Centers for Medicare & Medicaid Services wants physician input on developing direct provider contracting models to cover Medicare beneficiaries.

Under a direct provider contracting (DPC) arrangement, Medicare could pay physicians or physician groups a monthly fee to deliver a specific set of services to beneficiaries, who would gain greater access to the physicians. The physicians would be accountable for those Medicare patients’ costs and care quality.

zimmytws/gettyimages

CMS is looking at how to incorporate this concept into the Medicare ranks. On April 23, CMS issued a request for information (RFI) seeking input across a wide range of topics, including provider/state participation, beneficiary participation, payment, general model design, program integrity and beneficiary protection, and how such models would fit within the existing accountable care organization framework.

The RFI offered one possible vision on how a direct provider contracting model could work.

“Under a primary care–focused DPC model, CMS could enter into arrangements with primary care practices under which CMS would pay these participating practices a fixed per beneficiary per month (PBPM) payment to cover the primary care services the practice would be expected to furnish under the model, which may include office visits, certain office-based procedures, and other non–visit-based services covered under the physician fee schedule, and flexibility in how otherwise billable services are delivered,” the RFI states.

Physicians could also earn performance bonuses, depending on how the DPC is structured, through “performance-based incentives for total cost of care and quality.”

CMS noted it also “could test ways to reduce administrative burden though innovative changes to claims submission processes for services included in the PBPM payment under these models.”

 

 


The direct provider contracting idea grew out of a previous RFI issued in 2017 by CMS’s Center for Medicare and Medicaid Innovation to collect ideas on new ways to deliver patient-centered care. The agency released the more than 1,000 comments received from that request on the same day it issued the RFI on direct provider contracting.

In those comments, a number of physician groups offered support for a direct-contracting approach.

For example, the American Academy of Family Physicians wrote that it “sees continued growth and interest in family physicians adopting this practice model in all settings types, including rural and underserved communities.” And the AAFP suggested that the innovation center should work with DPC organizations to learn more about them.

The American College of Physicians reiterated its previous position that it “supports physician and patient choice of practice and delivery models that are accessible, ethical, and viable and that strengthen the patient-physician relationship.” But the ACP raised a number of issues that could impede access to care or result in lower quality care.

 

 


The American Medical Association offered support for “testing of models in which physicians have the ability to deliver more or different services to patients who need them and to be paid more for doing so.”

The AMA suggested that some of the models to be tested include allowing patients to contract directly with physicians, with Medicare paying its fee schedule rates and patients paying the difference; allowing patients to receive their care from DPC practices and get reimbursed by Medicare; or allowing “physicians to define a team of providers who will provide all of the treatment needed for an acute condition or management of a chronic condition, and then allowing patients who select the team to receive all of the services related to their condition from the team in return for a single predefined cost-sharing amount.”

Comments on the RFI are due May 25.

 

The Centers for Medicare & Medicaid Services wants physician input on developing direct provider contracting models to cover Medicare beneficiaries.

Under a direct provider contracting (DPC) arrangement, Medicare could pay physicians or physician groups a monthly fee to deliver a specific set of services to beneficiaries, who would gain greater access to the physicians. The physicians would be accountable for those Medicare patients’ costs and care quality.

zimmytws/gettyimages

CMS is looking at how to incorporate this concept into the Medicare ranks. On April 23, CMS issued a request for information (RFI) seeking input across a wide range of topics, including provider/state participation, beneficiary participation, payment, general model design, program integrity and beneficiary protection, and how such models would fit within the existing accountable care organization framework.

The RFI offered one possible vision on how a direct provider contracting model could work.

“Under a primary care–focused DPC model, CMS could enter into arrangements with primary care practices under which CMS would pay these participating practices a fixed per beneficiary per month (PBPM) payment to cover the primary care services the practice would be expected to furnish under the model, which may include office visits, certain office-based procedures, and other non–visit-based services covered under the physician fee schedule, and flexibility in how otherwise billable services are delivered,” the RFI states.

Physicians could also earn performance bonuses, depending on how the DPC is structured, through “performance-based incentives for total cost of care and quality.”

CMS noted it also “could test ways to reduce administrative burden though innovative changes to claims submission processes for services included in the PBPM payment under these models.”

 

 


The direct provider contracting idea grew out of a previous RFI issued in 2017 by CMS’s Center for Medicare and Medicaid Innovation to collect ideas on new ways to deliver patient-centered care. The agency released the more than 1,000 comments received from that request on the same day it issued the RFI on direct provider contracting.

In those comments, a number of physician groups offered support for a direct-contracting approach.

For example, the American Academy of Family Physicians wrote that it “sees continued growth and interest in family physicians adopting this practice model in all settings types, including rural and underserved communities.” And the AAFP suggested that the innovation center should work with DPC organizations to learn more about them.

The American College of Physicians reiterated its previous position that it “supports physician and patient choice of practice and delivery models that are accessible, ethical, and viable and that strengthen the patient-physician relationship.” But the ACP raised a number of issues that could impede access to care or result in lower quality care.

 

 


The American Medical Association offered support for “testing of models in which physicians have the ability to deliver more or different services to patients who need them and to be paid more for doing so.”

The AMA suggested that some of the models to be tested include allowing patients to contract directly with physicians, with Medicare paying its fee schedule rates and patients paying the difference; allowing patients to receive their care from DPC practices and get reimbursed by Medicare; or allowing “physicians to define a team of providers who will provide all of the treatment needed for an acute condition or management of a chronic condition, and then allowing patients who select the team to receive all of the services related to their condition from the team in return for a single predefined cost-sharing amount.”

Comments on the RFI are due May 25.

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Liquid nicotine for e-cigarettes may poison young children

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Abigail Cruz

 

Liquid nicotine for e-cigarettes poses a poisoning risk for young children, said Preethi Govindarajan of the Research Institute at Nationwide Children’s Hospital in Columbus, Ohio, and his associates.

Since January 2015, pediatric exposures to liquid nicotine have decreased, which may be attributable to legislation requiring child-resistant packaging for liquid nicotine containers and also greater public awareness of the risks associated with e-cigarette products, they noted.

Carpe89/ThinkStock
From January 2012 through April 2017, there were 8,269 liquid nicotine exposures reported among children younger 6 years to the National Poison Data System, with these incidents peaking in January 2015. The median age of exposure was 2 years, 84% of the children exposed were less than 3 years of age, and 55% of the children were boys. Of the exposures reported, 93% were because of ingestion. Fewer than 2% were admitted to the hospital. There was one death.

There was a significant increase in the monthly number of liquid nicotine exposures of 2,390% (P less than .001) from November 2012 through January 2015, and a significant decrease of 48% (P less than .001) from January 2015 through April 2017. “From August 2016 (175 exposures), the first month after the federal Child Nicotine Poisoning Prevention Act went into effect, to April 2017 (142 exposures), there was an 18.9% decrease in the number of monthly liquid nicotine exposures,” Mr. Govindarajan and his associates said.

“Child-resistant e-cigarette devices, use of flow restrictors on liquid nicotine containers, and regulations on e-cigarette liquid flavoring, labeling, and concentrations could further reduce the incidence of these exposures and the likelihood of serious medical outcomes when exposures do occur,” they concluded.

SOURCE: Govindarajan P et al. Pediatrics. 2018;141(5):e20173361.

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Abigail Cruz

 

Liquid nicotine for e-cigarettes poses a poisoning risk for young children, said Preethi Govindarajan of the Research Institute at Nationwide Children’s Hospital in Columbus, Ohio, and his associates.

Since January 2015, pediatric exposures to liquid nicotine have decreased, which may be attributable to legislation requiring child-resistant packaging for liquid nicotine containers and also greater public awareness of the risks associated with e-cigarette products, they noted.

Carpe89/ThinkStock
From January 2012 through April 2017, there were 8,269 liquid nicotine exposures reported among children younger 6 years to the National Poison Data System, with these incidents peaking in January 2015. The median age of exposure was 2 years, 84% of the children exposed were less than 3 years of age, and 55% of the children were boys. Of the exposures reported, 93% were because of ingestion. Fewer than 2% were admitted to the hospital. There was one death.

There was a significant increase in the monthly number of liquid nicotine exposures of 2,390% (P less than .001) from November 2012 through January 2015, and a significant decrease of 48% (P less than .001) from January 2015 through April 2017. “From August 2016 (175 exposures), the first month after the federal Child Nicotine Poisoning Prevention Act went into effect, to April 2017 (142 exposures), there was an 18.9% decrease in the number of monthly liquid nicotine exposures,” Mr. Govindarajan and his associates said.

“Child-resistant e-cigarette devices, use of flow restrictors on liquid nicotine containers, and regulations on e-cigarette liquid flavoring, labeling, and concentrations could further reduce the incidence of these exposures and the likelihood of serious medical outcomes when exposures do occur,” they concluded.

SOURCE: Govindarajan P et al. Pediatrics. 2018;141(5):e20173361.

 

Liquid nicotine for e-cigarettes poses a poisoning risk for young children, said Preethi Govindarajan of the Research Institute at Nationwide Children’s Hospital in Columbus, Ohio, and his associates.

Since January 2015, pediatric exposures to liquid nicotine have decreased, which may be attributable to legislation requiring child-resistant packaging for liquid nicotine containers and also greater public awareness of the risks associated with e-cigarette products, they noted.

Carpe89/ThinkStock
From January 2012 through April 2017, there were 8,269 liquid nicotine exposures reported among children younger 6 years to the National Poison Data System, with these incidents peaking in January 2015. The median age of exposure was 2 years, 84% of the children exposed were less than 3 years of age, and 55% of the children were boys. Of the exposures reported, 93% were because of ingestion. Fewer than 2% were admitted to the hospital. There was one death.

There was a significant increase in the monthly number of liquid nicotine exposures of 2,390% (P less than .001) from November 2012 through January 2015, and a significant decrease of 48% (P less than .001) from January 2015 through April 2017. “From August 2016 (175 exposures), the first month after the federal Child Nicotine Poisoning Prevention Act went into effect, to April 2017 (142 exposures), there was an 18.9% decrease in the number of monthly liquid nicotine exposures,” Mr. Govindarajan and his associates said.

“Child-resistant e-cigarette devices, use of flow restrictors on liquid nicotine containers, and regulations on e-cigarette liquid flavoring, labeling, and concentrations could further reduce the incidence of these exposures and the likelihood of serious medical outcomes when exposures do occur,” they concluded.

SOURCE: Govindarajan P et al. Pediatrics. 2018;141(5):e20173361.

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