Clinical Topics & News

Purpose 

To evaluate how various demographic factors impact the stage of cancer at diagnosis.

Background 

Laryngeal squamous cell carcinoma (SCC) is a common cancer with variable clinical presentation. While the probability of cure is high, more advanced tumors are less likely to be cured and more likely to have functional deficits from surgical treatment. Given the worsened prognosis of a later-stage diagnosis, it is important to understand what may contribute to a late presentation.

Method/Analysis

Using the National Cancer Database (NCDB), 73,330 patients were identified between 2004 and 2016 with laryngeal SCC. Early (stage 0 or I) vs late-stage (stage IV) cancers were compared based on demographic variables utilizing descriptive statistics, multivariate, and chi-square analyses on SPSS version 28 with a significance of P < .05.

Results 

Women were 27% more likely to have late-stage SCC than men. Black patients were 44% more likely to have late-stage SCC than White patients. No significant difference was found between Hispanic and non-Hispanic patients. Patients with private insurance, Medicare, or other government insurance were less likely (73%, 74%, and 62%, respectively) to have late-stage SCC compared to patients without insurance. Patients with Medicaid were 12% more likely to present later than the uninsured. Patients making $63,000 or greater were 23% less likely to have late-stage SCC than those making less than $38,000. Patients living in more educated areas (< 7% of adults had no high school degree) were 32% less likely to have late-stage SCC compared to less educated areas (> 21% of adults had no high school degree).

Conclusions/Implications

Patients who are Black, uninsured or on Medicaid, have low-socioeconomic status, and live in less educated areas have less favorable diagnoses than their counterparts. These data demonstrates inequities in health care and may lead to a better understanding of social determinants of health that can be used to advocate for improved access and quality of care.

Purpose 

To evaluate how various demographic factors impact the stage of cancer at diagnosis.

Background 

Laryngeal squamous cell carcinoma (SCC) is a common cancer with variable clinical presentation. While the probability of cure is high, more advanced tumors are less likely to be cured and more likely to have functional deficits from surgical treatment. Given the worsened prognosis of a later-stage diagnosis, it is important to understand what may contribute to a late presentation.

Method/Analysis

Using the National Cancer Database (NCDB), 73,330 patients were identified between 2004 and 2016 with laryngeal SCC. Early (stage 0 or I) vs late-stage (stage IV) cancers were compared based on demographic variables utilizing descriptive statistics, multivariate, and chi-square analyses on SPSS version 28 with a significance of P < .05.

Results 

Women were 27% more likely to have late-stage SCC than men. Black patients were 44% more likely to have late-stage SCC than White patients. No significant difference was found between Hispanic and non-Hispanic patients. Patients with private insurance, Medicare, or other government insurance were less likely (73%, 74%, and 62%, respectively) to have late-stage SCC compared to patients without insurance. Patients with Medicaid were 12% more likely to present later than the uninsured. Patients making $63,000 or greater were 23% less likely to have late-stage SCC than those making less than $38,000. Patients living in more educated areas (< 7% of adults had no high school degree) were 32% less likely to have late-stage SCC compared to less educated areas (> 21% of adults had no high school degree).

Conclusions/Implications

Patients who are Black, uninsured or on Medicaid, have low-socioeconomic status, and live in less educated areas have less favorable diagnoses than their counterparts. These data demonstrates inequities in health care and may lead to a better understanding of social determinants of health that can be used to advocate for improved access and quality of care.

Purpose 

To evaluate how various demographic factors impact the stage of cancer at diagnosis.

Background 

Laryngeal squamous cell carcinoma (SCC) is a common cancer with variable clinical presentation. While the probability of cure is high, more advanced tumors are less likely to be cured and more likely to have functional deficits from surgical treatment. Given the worsened prognosis of a later-stage diagnosis, it is important to understand what may contribute to a late presentation.

Method/Analysis

Using the National Cancer Database (NCDB), 73,330 patients were identified between 2004 and 2016 with laryngeal SCC. Early (stage 0 or I) vs late-stage (stage IV) cancers were compared based on demographic variables utilizing descriptive statistics, multivariate, and chi-square analyses on SPSS version 28 with a significance of P < .05.

Results 

Women were 27% more likely to have late-stage SCC than men. Black patients were 44% more likely to have late-stage SCC than White patients. No significant difference was found between Hispanic and non-Hispanic patients. Patients with private insurance, Medicare, or other government insurance were less likely (73%, 74%, and 62%, respectively) to have late-stage SCC compared to patients without insurance. Patients with Medicaid were 12% more likely to present later than the uninsured. Patients making $63,000 or greater were 23% less likely to have late-stage SCC than those making less than $38,000. Patients living in more educated areas (< 7% of adults had no high school degree) were 32% less likely to have late-stage SCC compared to less educated areas (> 21% of adults had no high school degree).

Conclusions/Implications

Patients who are Black, uninsured or on Medicaid, have low-socioeconomic status, and live in less educated areas have less favorable diagnoses than their counterparts. These data demonstrates inequities in health care and may lead to a better understanding of social determinants of health that can be used to advocate for improved access and quality of care.

Introduction

Idiopathic multicentric Castleman disease (iMCD) cases with thrombocytopenia (T), anasarca (A), Fever (F), reticulin fibrosis (R), and organomegaly (O) are considered a subtype of iMCS called TAFRO syndrome. It is a rare syndrome characterized by an acute clinical course and a poor prognosis. The optimal management of TAFRO syndrome is unclear. Treatment is predicated on case reports, which have used an assortment of agents including corticosteroids, cyclosporin, rituximab, and/or anti-IL-6 therapies such as tocilizumab or siltuximab.

Case Report 

A 73-year-old Japanese man recently diagnosed with iMCD but not currently on treatment presented with a 3-week history of worsening lower extremity edema and abdominal distension. On exam, he had anasarca and was also found to be anuric. Diagnostic testing was notable for hemoglobin 10.9 g/dL, platelets 42 K/uL, potassium 5.7 mmol/L, creatinine 3.7, albumin 2.4 g/dL, troponin 0.1, BNP 805, and Echocardiogram demonstrating an ejection fraction of 40% to 50%. Imaging revealed pleural effusion, splenomegaly and large volume ascites while paracentesis confirmed transudative ascitic fluid. He failed a trial of IV diuresis and was started on dialysis for refractory hyperkalemia. Upon review, his prior bone marrow had mild reticulin fibrosis, and additional lab testing showed an elevated hs-CRP 101 mg/dL and IL-6 of 4.8 pg/mL. His presentation fit multiple criteria for TAFRO including thrombocytopenia, anasarca, fibrosis in bone marrow, renal failure and organomegaly. He was started on weekly rituximab for 4 doses, as well as daily dexamethasone 40 mg for 4 days followed by a planned 6-week prednisone taper. Within a week his urine output began to improve, creatinine improved to 2 and he no longer needed dialysis.
Despite improvement in his renal function, he had progressive anasarca, fatigue and appetite loss over the next three weeks. He was given one dose of siltuximab as salvage therapy, but due to worsening quality of life, he transitioned to comfort care shortly thereafter with eventual demise.

Discussion

Determination of iMCD-TAFRO syndrome requires high clinical suspicion from clinicians to enable early treatment. This case report illustrates the need for early recognition and aggressive treatment to improve outcomes in patients with this deadly disease.

Introduction

Idiopathic multicentric Castleman disease (iMCD) cases with thrombocytopenia (T), anasarca (A), Fever (F), reticulin fibrosis (R), and organomegaly (O) are considered a subtype of iMCS called TAFRO syndrome. It is a rare syndrome characterized by an acute clinical course and a poor prognosis. The optimal management of TAFRO syndrome is unclear. Treatment is predicated on case reports, which have used an assortment of agents including corticosteroids, cyclosporin, rituximab, and/or anti-IL-6 therapies such as tocilizumab or siltuximab.

Case Report 

A 73-year-old Japanese man recently diagnosed with iMCD but not currently on treatment presented with a 3-week history of worsening lower extremity edema and abdominal distension. On exam, he had anasarca and was also found to be anuric. Diagnostic testing was notable for hemoglobin 10.9 g/dL, platelets 42 K/uL, potassium 5.7 mmol/L, creatinine 3.7, albumin 2.4 g/dL, troponin 0.1, BNP 805, and Echocardiogram demonstrating an ejection fraction of 40% to 50%. Imaging revealed pleural effusion, splenomegaly and large volume ascites while paracentesis confirmed transudative ascitic fluid. He failed a trial of IV diuresis and was started on dialysis for refractory hyperkalemia. Upon review, his prior bone marrow had mild reticulin fibrosis, and additional lab testing showed an elevated hs-CRP 101 mg/dL and IL-6 of 4.8 pg/mL. His presentation fit multiple criteria for TAFRO including thrombocytopenia, anasarca, fibrosis in bone marrow, renal failure and organomegaly. He was started on weekly rituximab for 4 doses, as well as daily dexamethasone 40 mg for 4 days followed by a planned 6-week prednisone taper. Within a week his urine output began to improve, creatinine improved to 2 and he no longer needed dialysis.
Despite improvement in his renal function, he had progressive anasarca, fatigue and appetite loss over the next three weeks. He was given one dose of siltuximab as salvage therapy, but due to worsening quality of life, he transitioned to comfort care shortly thereafter with eventual demise.

Discussion

Determination of iMCD-TAFRO syndrome requires high clinical suspicion from clinicians to enable early treatment. This case report illustrates the need for early recognition and aggressive treatment to improve outcomes in patients with this deadly disease.

Introduction

Idiopathic multicentric Castleman disease (iMCD) cases with thrombocytopenia (T), anasarca (A), Fever (F), reticulin fibrosis (R), and organomegaly (O) are considered a subtype of iMCS called TAFRO syndrome. It is a rare syndrome characterized by an acute clinical course and a poor prognosis. The optimal management of TAFRO syndrome is unclear. Treatment is predicated on case reports, which have used an assortment of agents including corticosteroids, cyclosporin, rituximab, and/or anti-IL-6 therapies such as tocilizumab or siltuximab.

Case Report 

A 73-year-old Japanese man recently diagnosed with iMCD but not currently on treatment presented with a 3-week history of worsening lower extremity edema and abdominal distension. On exam, he had anasarca and was also found to be anuric. Diagnostic testing was notable for hemoglobin 10.9 g/dL, platelets 42 K/uL, potassium 5.7 mmol/L, creatinine 3.7, albumin 2.4 g/dL, troponin 0.1, BNP 805, and Echocardiogram demonstrating an ejection fraction of 40% to 50%. Imaging revealed pleural effusion, splenomegaly and large volume ascites while paracentesis confirmed transudative ascitic fluid. He failed a trial of IV diuresis and was started on dialysis for refractory hyperkalemia. Upon review, his prior bone marrow had mild reticulin fibrosis, and additional lab testing showed an elevated hs-CRP 101 mg/dL and IL-6 of 4.8 pg/mL. His presentation fit multiple criteria for TAFRO including thrombocytopenia, anasarca, fibrosis in bone marrow, renal failure and organomegaly. He was started on weekly rituximab for 4 doses, as well as daily dexamethasone 40 mg for 4 days followed by a planned 6-week prednisone taper. Within a week his urine output began to improve, creatinine improved to 2 and he no longer needed dialysis.
Despite improvement in his renal function, he had progressive anasarca, fatigue and appetite loss over the next three weeks. He was given one dose of siltuximab as salvage therapy, but due to worsening quality of life, he transitioned to comfort care shortly thereafter with eventual demise.

Discussion

Determination of iMCD-TAFRO syndrome requires high clinical suspicion from clinicians to enable early treatment. This case report illustrates the need for early recognition and aggressive treatment to improve outcomes in patients with this deadly disease.

Background

Schnitzler syndrome is a rare, auto-inflammatory syndrome associated with IgM monoclonal gammopathy that manifests as a non-pruritic urticarial rash. Patients may also have intermittent fevers, lymphadenopathy, bone pain, and arthralgias. Given its rarity and nonspecific presentation, its diagnosis requires a high index of clinical suspicion. Herein, we describe a case of a patient with a 2-year history of urticarial rash who then developed fevers, arthralgias, neutrophilia, and weight loss. He was ultimately found to have a diagnosis of Waldenstrom Macroglobulinemia (WM)/Lymphoplasmacytic Lymphoma (LPL), in the context of Schnitzler Syndrome.

Case Report 

A 74-year-old traveling veteran presenting with fatigue, weight loss, arthralgias and rash was found to have anemia and leukocytosis concerning an occult hematologic malignancy. On exam, his rash appeared classically urticarial. He described the rash as nonpruritic and ‘waxing and waning’ for ~18 months. Following the rash, he developed drenching night sweats which progressively worsened over the last 4 months. This was accompanied by fatigue and arthralgias. On review of his labs, he had a normocytic anemia, thrombocytosis and progressive neutrophilia of approximately 20,000 over the last 4 months. Peripheral blood smear was remarkable for atypical lymphocytes. Peripheral blood flow cytometry revealed a small monoclonal CD5-/CD10- B-cell population of uncertain significance. His total serum IgM level was > 1000 mg/dL and serum protein electrophoresis with IFE confirmed a monoclonal IgM gammopathy with M-spike of 0.99 g/dL.

Results 

A bone marrow biopsy showed a hypercellular marrow with LPL comprising 50%-60% of the cellularity. It was also notable for grade 1/3 reticulin fibrosis and mild megakaryocytic aty WM/LPL can have a heterogeneous presentation. Urticarial rash, constitutional symptoms, joint pain, and neutrophilia should raise suspicion for Schnitzler syndrome, especially in conjunction with IgM monoclonal gammopathy. This rare syndrome is imperative to consider because it can be treated quickly with high efficacy with IL-1 antagonism. Anakinra is clinically effective for symptom management while awaiting primary treatment for underlying LPL.

Background

Schnitzler syndrome is a rare, auto-inflammatory syndrome associated with IgM monoclonal gammopathy that manifests as a non-pruritic urticarial rash. Patients may also have intermittent fevers, lymphadenopathy, bone pain, and arthralgias. Given its rarity and nonspecific presentation, its diagnosis requires a high index of clinical suspicion. Herein, we describe a case of a patient with a 2-year history of urticarial rash who then developed fevers, arthralgias, neutrophilia, and weight loss. He was ultimately found to have a diagnosis of Waldenstrom Macroglobulinemia (WM)/Lymphoplasmacytic Lymphoma (LPL), in the context of Schnitzler Syndrome.

Case Report 

A 74-year-old traveling veteran presenting with fatigue, weight loss, arthralgias and rash was found to have anemia and leukocytosis concerning an occult hematologic malignancy. On exam, his rash appeared classically urticarial. He described the rash as nonpruritic and ‘waxing and waning’ for ~18 months. Following the rash, he developed drenching night sweats which progressively worsened over the last 4 months. This was accompanied by fatigue and arthralgias. On review of his labs, he had a normocytic anemia, thrombocytosis and progressive neutrophilia of approximately 20,000 over the last 4 months. Peripheral blood smear was remarkable for atypical lymphocytes. Peripheral blood flow cytometry revealed a small monoclonal CD5-/CD10- B-cell population of uncertain significance. His total serum IgM level was > 1000 mg/dL and serum protein electrophoresis with IFE confirmed a monoclonal IgM gammopathy with M-spike of 0.99 g/dL.

Results 

A bone marrow biopsy showed a hypercellular marrow with LPL comprising 50%-60% of the cellularity. It was also notable for grade 1/3 reticulin fibrosis and mild megakaryocytic aty WM/LPL can have a heterogeneous presentation. Urticarial rash, constitutional symptoms, joint pain, and neutrophilia should raise suspicion for Schnitzler syndrome, especially in conjunction with IgM monoclonal gammopathy. This rare syndrome is imperative to consider because it can be treated quickly with high efficacy with IL-1 antagonism. Anakinra is clinically effective for symptom management while awaiting primary treatment for underlying LPL.

Background

Schnitzler syndrome is a rare, auto-inflammatory syndrome associated with IgM monoclonal gammopathy that manifests as a non-pruritic urticarial rash. Patients may also have intermittent fevers, lymphadenopathy, bone pain, and arthralgias. Given its rarity and nonspecific presentation, its diagnosis requires a high index of clinical suspicion. Herein, we describe a case of a patient with a 2-year history of urticarial rash who then developed fevers, arthralgias, neutrophilia, and weight loss. He was ultimately found to have a diagnosis of Waldenstrom Macroglobulinemia (WM)/Lymphoplasmacytic Lymphoma (LPL), in the context of Schnitzler Syndrome.

Case Report 

A 74-year-old traveling veteran presenting with fatigue, weight loss, arthralgias and rash was found to have anemia and leukocytosis concerning an occult hematologic malignancy. On exam, his rash appeared classically urticarial. He described the rash as nonpruritic and ‘waxing and waning’ for ~18 months. Following the rash, he developed drenching night sweats which progressively worsened over the last 4 months. This was accompanied by fatigue and arthralgias. On review of his labs, he had a normocytic anemia, thrombocytosis and progressive neutrophilia of approximately 20,000 over the last 4 months. Peripheral blood smear was remarkable for atypical lymphocytes. Peripheral blood flow cytometry revealed a small monoclonal CD5-/CD10- B-cell population of uncertain significance. His total serum IgM level was > 1000 mg/dL and serum protein electrophoresis with IFE confirmed a monoclonal IgM gammopathy with M-spike of 0.99 g/dL.

Results 

A bone marrow biopsy showed a hypercellular marrow with LPL comprising 50%-60% of the cellularity. It was also notable for grade 1/3 reticulin fibrosis and mild megakaryocytic aty WM/LPL can have a heterogeneous presentation. Urticarial rash, constitutional symptoms, joint pain, and neutrophilia should raise suspicion for Schnitzler syndrome, especially in conjunction with IgM monoclonal gammopathy. This rare syndrome is imperative to consider because it can be treated quickly with high efficacy with IL-1 antagonism. Anakinra is clinically effective for symptom management while awaiting primary treatment for underlying LPL.

Study Purpose/Background

Malignant mixed Mullerian tumor (MMMT), known as carcinosarcoma of the uterus, is a rare tumor consisting of malignant epithelial and mesenchymal components. Palliative care (PC) has been shown to enhance quality of life and improve outcomes in patients with advanced or incurable cancer. Patients with MMMT may benefit from PC. The project’s objective is to describe disparities in PC utilization among MMMT patients using the National Cancer Database (NCDB).

Methods/Design

A total of 14,085 patients, diagnosed with MMMT, were identified utilizing the NCDB ICD-O-3 histology code 8950. Demographic factors (race, income, facility type, insurance, geographic location, grade, and Charlson-Deyo comorbidity score) were studied in relation to the receipt of PC using multivariate logistic regression.

Results 

3.10% of the study cohort received PC (437/14085). Participants with a median income of ≥ $63,000 (2.74%) were less likely to receive PC than participants making < $38,000 (3.93%), P = .049. Participants treated at an academic/research program (2.48%) were less likely to receive PC compared to patients treated at a community cancer program (4.44%), P = .023. Those with private insurance (2.50%), were less likely to receive PC than those with no insurance (3.56%), P = .032. Participants who received treatment at a facility located in the South Atlantic (2.25%), East North Central (3.11%), West South Central (2.73%) or Pacific (1.36%), were less likely to receive PC than patients who received treatment at a New England facility (4.42%), P < .001, P = .031, P = .017, and P < .001, respectively. Those with tumors that were undifferentiated, anaplastic (3.52%) were more likely to receive PC than those with well-differentiated tumors (1.01%), P = .040.

Conclusions/Implications

PC is underutilized in patients with private insurance, received treatment at an academic/research program, had well-differentiated tumors, and were in the South Atlantic, East North Central, West South Central, and Pacific regions. By highlighting disparities that exist, our study can aid clinicians in addressing PC underutilization to help provide more comprehensive care for patients.

Study Purpose/Background

Malignant mixed Mullerian tumor (MMMT), known as carcinosarcoma of the uterus, is a rare tumor consisting of malignant epithelial and mesenchymal components. Palliative care (PC) has been shown to enhance quality of life and improve outcomes in patients with advanced or incurable cancer. Patients with MMMT may benefit from PC. The project’s objective is to describe disparities in PC utilization among MMMT patients using the National Cancer Database (NCDB).

Methods/Design

A total of 14,085 patients, diagnosed with MMMT, were identified utilizing the NCDB ICD-O-3 histology code 8950. Demographic factors (race, income, facility type, insurance, geographic location, grade, and Charlson-Deyo comorbidity score) were studied in relation to the receipt of PC using multivariate logistic regression.

Results 

3.10% of the study cohort received PC (437/14085). Participants with a median income of ≥ $63,000 (2.74%) were less likely to receive PC than participants making < $38,000 (3.93%), P = .049. Participants treated at an academic/research program (2.48%) were less likely to receive PC compared to patients treated at a community cancer program (4.44%), P = .023. Those with private insurance (2.50%), were less likely to receive PC than those with no insurance (3.56%), P = .032. Participants who received treatment at a facility located in the South Atlantic (2.25%), East North Central (3.11%), West South Central (2.73%) or Pacific (1.36%), were less likely to receive PC than patients who received treatment at a New England facility (4.42%), P < .001, P = .031, P = .017, and P < .001, respectively. Those with tumors that were undifferentiated, anaplastic (3.52%) were more likely to receive PC than those with well-differentiated tumors (1.01%), P = .040.

Conclusions/Implications

PC is underutilized in patients with private insurance, received treatment at an academic/research program, had well-differentiated tumors, and were in the South Atlantic, East North Central, West South Central, and Pacific regions. By highlighting disparities that exist, our study can aid clinicians in addressing PC underutilization to help provide more comprehensive care for patients.

Study Purpose/Background

Malignant mixed Mullerian tumor (MMMT), known as carcinosarcoma of the uterus, is a rare tumor consisting of malignant epithelial and mesenchymal components. Palliative care (PC) has been shown to enhance quality of life and improve outcomes in patients with advanced or incurable cancer. Patients with MMMT may benefit from PC. The project’s objective is to describe disparities in PC utilization among MMMT patients using the National Cancer Database (NCDB).

Methods/Design

A total of 14,085 patients, diagnosed with MMMT, were identified utilizing the NCDB ICD-O-3 histology code 8950. Demographic factors (race, income, facility type, insurance, geographic location, grade, and Charlson-Deyo comorbidity score) were studied in relation to the receipt of PC using multivariate logistic regression.

Results 

3.10% of the study cohort received PC (437/14085). Participants with a median income of ≥ $63,000 (2.74%) were less likely to receive PC than participants making < $38,000 (3.93%), P = .049. Participants treated at an academic/research program (2.48%) were less likely to receive PC compared to patients treated at a community cancer program (4.44%), P = .023. Those with private insurance (2.50%), were less likely to receive PC than those with no insurance (3.56%), P = .032. Participants who received treatment at a facility located in the South Atlantic (2.25%), East North Central (3.11%), West South Central (2.73%) or Pacific (1.36%), were less likely to receive PC than patients who received treatment at a New England facility (4.42%), P < .001, P = .031, P = .017, and P < .001, respectively. Those with tumors that were undifferentiated, anaplastic (3.52%) were more likely to receive PC than those with well-differentiated tumors (1.01%), P = .040.

Conclusions/Implications

PC is underutilized in patients with private insurance, received treatment at an academic/research program, had well-differentiated tumors, and were in the South Atlantic, East North Central, West South Central, and Pacific regions. By highlighting disparities that exist, our study can aid clinicians in addressing PC underutilization to help provide more comprehensive care for patients.

Clinical Presentation

A 58-year-old male was diagnosed 6 years ago with stage IV clear cell renal carcinoma (multiple lung nodules and mediastinal adenopathy). He was offered sunitinib, a tyrosine kinase inhibitor, and achieved a partial response with stable disease. Five years later his scans showed worsening disease. Cabozantinib was offered but was poorly tolerated. He tried ipilimumab plus nivolumab but ipilimumab was dropped after 4 cycles due to diarrhea. His scans improved with 4 more cycles of nivolumab but he had to stop immunotherapy due to hypophysitis, diarrhea, and severe jaw pain. He received a COVID-19 booster vaccine and noticed profound fatigue and anorexia soon after. Over 8 weeks he lost 56 lbs (267 to 211 lb). Relapse was suspected but PET CT showed complete resolution of his lung nodules and multiple areas of adenopathy. Asymptomatic and in remission 6 months after vaccination.

Relevant Literature 

Clear cell renal carcinoma is resistant to standard chemotherapy/radiation, which usually offers partial responses. Complete remissions are few. Low glycemic diets in animal models have anticancer activity. HIV causes B cell apoptosis. Coxsackievirus A21 oncolytic properties lyse myeloma and CD138+ plasma cells via intercellular adhesion molecule interaction. SARS-CoV-2 (COVID 19) proteins have oncolytic properties.

Intervention

The patient eliminated sugary food from his diet 6 years ago. Stopped bread 2 years ago. Sunitinib 37.5 mg daily × 5 years. Cabozantinib—poorly tolerated. Ipilimumab + nivolumab × 4 cycles followed by nivolumab × 4 cycles. Stopped treatment; immune side effects. COVID-19 booster.

Outcome

15 lb weight loss due to a low glycemic diet, which began since diagnosis. After 4 years he stopped eating bread. After COVID-19 vaccine had a rapid 56 lb. weight loss, fatigue, and nausea over 8 weeks. No evidence of disease. Asymptomatic, off therapy, weight is ideal (219 lb) 6 months after the vaccine.

Implications for Practice 

Effects of SARS-CoV-2 (COVID-19) on cancers remain unknown. A few case reports of cancer remissions after infections are emerging. This is the first case of complete remission after COVID-19 vaccination in a patient on immunotherapy/low glycemic diet. Research is needed to study the contribution of a COVID-19 inflammatory response.

Clinical Presentation

A 58-year-old male was diagnosed 6 years ago with stage IV clear cell renal carcinoma (multiple lung nodules and mediastinal adenopathy). He was offered sunitinib, a tyrosine kinase inhibitor, and achieved a partial response with stable disease. Five years later his scans showed worsening disease. Cabozantinib was offered but was poorly tolerated. He tried ipilimumab plus nivolumab but ipilimumab was dropped after 4 cycles due to diarrhea. His scans improved with 4 more cycles of nivolumab but he had to stop immunotherapy due to hypophysitis, diarrhea, and severe jaw pain. He received a COVID-19 booster vaccine and noticed profound fatigue and anorexia soon after. Over 8 weeks he lost 56 lbs (267 to 211 lb). Relapse was suspected but PET CT showed complete resolution of his lung nodules and multiple areas of adenopathy. Asymptomatic and in remission 6 months after vaccination.

Relevant Literature 

Clear cell renal carcinoma is resistant to standard chemotherapy/radiation, which usually offers partial responses. Complete remissions are few. Low glycemic diets in animal models have anticancer activity. HIV causes B cell apoptosis. Coxsackievirus A21 oncolytic properties lyse myeloma and CD138+ plasma cells via intercellular adhesion molecule interaction. SARS-CoV-2 (COVID 19) proteins have oncolytic properties.

Intervention

The patient eliminated sugary food from his diet 6 years ago. Stopped bread 2 years ago. Sunitinib 37.5 mg daily × 5 years. Cabozantinib—poorly tolerated. Ipilimumab + nivolumab × 4 cycles followed by nivolumab × 4 cycles. Stopped treatment; immune side effects. COVID-19 booster.

Outcome

15 lb weight loss due to a low glycemic diet, which began since diagnosis. After 4 years he stopped eating bread. After COVID-19 vaccine had a rapid 56 lb. weight loss, fatigue, and nausea over 8 weeks. No evidence of disease. Asymptomatic, off therapy, weight is ideal (219 lb) 6 months after the vaccine.

Implications for Practice 

Effects of SARS-CoV-2 (COVID-19) on cancers remain unknown. A few case reports of cancer remissions after infections are emerging. This is the first case of complete remission after COVID-19 vaccination in a patient on immunotherapy/low glycemic diet. Research is needed to study the contribution of a COVID-19 inflammatory response.

Clinical Presentation

A 58-year-old male was diagnosed 6 years ago with stage IV clear cell renal carcinoma (multiple lung nodules and mediastinal adenopathy). He was offered sunitinib, a tyrosine kinase inhibitor, and achieved a partial response with stable disease. Five years later his scans showed worsening disease. Cabozantinib was offered but was poorly tolerated. He tried ipilimumab plus nivolumab but ipilimumab was dropped after 4 cycles due to diarrhea. His scans improved with 4 more cycles of nivolumab but he had to stop immunotherapy due to hypophysitis, diarrhea, and severe jaw pain. He received a COVID-19 booster vaccine and noticed profound fatigue and anorexia soon after. Over 8 weeks he lost 56 lbs (267 to 211 lb). Relapse was suspected but PET CT showed complete resolution of his lung nodules and multiple areas of adenopathy. Asymptomatic and in remission 6 months after vaccination.

Relevant Literature 

Clear cell renal carcinoma is resistant to standard chemotherapy/radiation, which usually offers partial responses. Complete remissions are few. Low glycemic diets in animal models have anticancer activity. HIV causes B cell apoptosis. Coxsackievirus A21 oncolytic properties lyse myeloma and CD138+ plasma cells via intercellular adhesion molecule interaction. SARS-CoV-2 (COVID 19) proteins have oncolytic properties.

Intervention

The patient eliminated sugary food from his diet 6 years ago. Stopped bread 2 years ago. Sunitinib 37.5 mg daily × 5 years. Cabozantinib—poorly tolerated. Ipilimumab + nivolumab × 4 cycles followed by nivolumab × 4 cycles. Stopped treatment; immune side effects. COVID-19 booster.

Outcome

15 lb weight loss due to a low glycemic diet, which began since diagnosis. After 4 years he stopped eating bread. After COVID-19 vaccine had a rapid 56 lb. weight loss, fatigue, and nausea over 8 weeks. No evidence of disease. Asymptomatic, off therapy, weight is ideal (219 lb) 6 months after the vaccine.

Implications for Practice 

Effects of SARS-CoV-2 (COVID-19) on cancers remain unknown. A few case reports of cancer remissions after infections are emerging. This is the first case of complete remission after COVID-19 vaccination in a patient on immunotherapy/low glycemic diet. Research is needed to study the contribution of a COVID-19 inflammatory response.

Background

There is unequal access to subspecialty oncology expertise across the Veterans Affairs (VA) network. To address this need, the VA established National TeleOncology (NTO), which provides multiple virtual services (asynchronous [electronic consult] and synchronous [phone, video to home, video to facility]) to over 20 VA sites. Beyond these care modalities, a virtual tumor board was conceived to provide a forum for multidisciplinary review of patient cases. We describe the creation of the first NTO virtual tumor board, encompassing malignant hematology diagnoses.

Observations

Tumor boards are considered a standard of care. While challenging to quantify nationally, multiple single institution experiences have established the importance of tumor boards across different measures. A panel of stakeholders were convened to discuss the creation of a virtual tumor board. Best practices and standard operating procedures were created based on guidance from relevant literature and internal VA experience. Participants from specialties including medical oncology, surgical oncology, radiology, pathology, transplant, and palliative care were engaged from eight different VA medical centers across the nation. On March 2, 2022, the initial tumor board was held allowing for synchronous virtual review of patient pathology and imaging. Thus far 6 tumor boards have been convened, reviewing 11 patients originating from 6 different VA sites.

Results

A participant survey was conducted after 4 sessions, which indicated that all who completed the survey (n = 9) found the sessions beneficial or somewhat beneficial, and 55% found the sessions highly applicable to their practice. The most recent tumor board had 33 participants (physicians, nurses, advanced practice practitioners, and pharmacists).

Conclusions

The establishment of a national VA tumor board represents a novel approach to the review of oncology cases across the VA network. The goal of this tumor board is to leverage the diverse knowledge base that exists within the VA to deliver equitable care regardless of veteran location. Along with improving our general understanding of tumor board application, we believe that the NTO tumor board establishes a unique forum for additional tumor types, continued medical education opportunities, and the review of VA clinical trial opportunities.

Background

There is unequal access to subspecialty oncology expertise across the Veterans Affairs (VA) network. To address this need, the VA established National TeleOncology (NTO), which provides multiple virtual services (asynchronous [electronic consult] and synchronous [phone, video to home, video to facility]) to over 20 VA sites. Beyond these care modalities, a virtual tumor board was conceived to provide a forum for multidisciplinary review of patient cases. We describe the creation of the first NTO virtual tumor board, encompassing malignant hematology diagnoses.

Observations

Tumor boards are considered a standard of care. While challenging to quantify nationally, multiple single institution experiences have established the importance of tumor boards across different measures. A panel of stakeholders were convened to discuss the creation of a virtual tumor board. Best practices and standard operating procedures were created based on guidance from relevant literature and internal VA experience. Participants from specialties including medical oncology, surgical oncology, radiology, pathology, transplant, and palliative care were engaged from eight different VA medical centers across the nation. On March 2, 2022, the initial tumor board was held allowing for synchronous virtual review of patient pathology and imaging. Thus far 6 tumor boards have been convened, reviewing 11 patients originating from 6 different VA sites.

Results

A participant survey was conducted after 4 sessions, which indicated that all who completed the survey (n = 9) found the sessions beneficial or somewhat beneficial, and 55% found the sessions highly applicable to their practice. The most recent tumor board had 33 participants (physicians, nurses, advanced practice practitioners, and pharmacists).

Conclusions

The establishment of a national VA tumor board represents a novel approach to the review of oncology cases across the VA network. The goal of this tumor board is to leverage the diverse knowledge base that exists within the VA to deliver equitable care regardless of veteran location. Along with improving our general understanding of tumor board application, we believe that the NTO tumor board establishes a unique forum for additional tumor types, continued medical education opportunities, and the review of VA clinical trial opportunities.

Background

There is unequal access to subspecialty oncology expertise across the Veterans Affairs (VA) network. To address this need, the VA established National TeleOncology (NTO), which provides multiple virtual services (asynchronous [electronic consult] and synchronous [phone, video to home, video to facility]) to over 20 VA sites. Beyond these care modalities, a virtual tumor board was conceived to provide a forum for multidisciplinary review of patient cases. We describe the creation of the first NTO virtual tumor board, encompassing malignant hematology diagnoses.

Observations

Tumor boards are considered a standard of care. While challenging to quantify nationally, multiple single institution experiences have established the importance of tumor boards across different measures. A panel of stakeholders were convened to discuss the creation of a virtual tumor board. Best practices and standard operating procedures were created based on guidance from relevant literature and internal VA experience. Participants from specialties including medical oncology, surgical oncology, radiology, pathology, transplant, and palliative care were engaged from eight different VA medical centers across the nation. On March 2, 2022, the initial tumor board was held allowing for synchronous virtual review of patient pathology and imaging. Thus far 6 tumor boards have been convened, reviewing 11 patients originating from 6 different VA sites.

Results

A participant survey was conducted after 4 sessions, which indicated that all who completed the survey (n = 9) found the sessions beneficial or somewhat beneficial, and 55% found the sessions highly applicable to their practice. The most recent tumor board had 33 participants (physicians, nurses, advanced practice practitioners, and pharmacists).

Conclusions

The establishment of a national VA tumor board represents a novel approach to the review of oncology cases across the VA network. The goal of this tumor board is to leverage the diverse knowledge base that exists within the VA to deliver equitable care regardless of veteran location. Along with improving our general understanding of tumor board application, we believe that the NTO tumor board establishes a unique forum for additional tumor types, continued medical education opportunities, and the review of VA clinical trial opportunities.

Background

Since inception, the Veterans Affairs (VA) National TeleOncology (NTO) service has monitored clinical operations through data tools produced by the Veterans Health Administration Support Service Center (VSSC). Unfortunately, pertinent data are spread across multiple reports, making it difficult to continually harmonize needed information. Further, the VSSC does not account for NTO’s hub and spoke clinical model, leading to inaccuracies when attempting to analyze unique encounters. To address these challenges, NTO partnered with the VA Salt Lake City Health Care System Informatics, Decision-Enhancement, and Analytic Sciences Center (IDEAS) to develop an informatics architecture and frontend NTO Clinical Operations Dashboard (NCOD). Here, we summarize our dashboard development process and the finalized key reporting components of the NCOD.

Methods

The VA Corporate Data Warehouse (CDW) serves as the primary data source for the NCOD. SQL Server Integration Services was used to build the backend data architecture. Data from the CDW were isolated into a staging data mart for reporting purposes using an extract, transform, load (ETL) approach. The frontend user interface was developed using Power BI. We used a participatory approach1 in determining reporting requirements. Stakeholders included the IDEAS dashboard development team and potential end users from NTO, including leadership, program managers, support assistants, and telehealth coordinators.

Results

The NCOD ETL is scheduled to refresh the data nightly to provide end users with a near real-time experience. The NCOD is comprised of the following four data views: clinic availability, team productivity, patient summary, and encounter summary. The clinic availability view summarizes clinic capacity, no shows, overbookings, and percent utilization. Relative value unit- based productivity is summarized in the team productivity view. The patient summary view presents aggregated data for veterans served by NTO, including geographic distribution, with patient-level drill down displaying demographics, cancer characteristics, and treatment history. Lastly, the encounter view displays utilization trends by modality, while accurately accounting for the hub and spoke clinical model.

Conclusions

An informatics architecture and frontend information display that is capable of synthesizing EHR data into a consumable format has been pivotal in obtaining accurate and timely insight into the demand and capacity of services provided by NTO.

Background

Since inception, the Veterans Affairs (VA) National TeleOncology (NTO) service has monitored clinical operations through data tools produced by the Veterans Health Administration Support Service Center (VSSC). Unfortunately, pertinent data are spread across multiple reports, making it difficult to continually harmonize needed information. Further, the VSSC does not account for NTO’s hub and spoke clinical model, leading to inaccuracies when attempting to analyze unique encounters. To address these challenges, NTO partnered with the VA Salt Lake City Health Care System Informatics, Decision-Enhancement, and Analytic Sciences Center (IDEAS) to develop an informatics architecture and frontend NTO Clinical Operations Dashboard (NCOD). Here, we summarize our dashboard development process and the finalized key reporting components of the NCOD.

Methods

The VA Corporate Data Warehouse (CDW) serves as the primary data source for the NCOD. SQL Server Integration Services was used to build the backend data architecture. Data from the CDW were isolated into a staging data mart for reporting purposes using an extract, transform, load (ETL) approach. The frontend user interface was developed using Power BI. We used a participatory approach1 in determining reporting requirements. Stakeholders included the IDEAS dashboard development team and potential end users from NTO, including leadership, program managers, support assistants, and telehealth coordinators.

Results

The NCOD ETL is scheduled to refresh the data nightly to provide end users with a near real-time experience. The NCOD is comprised of the following four data views: clinic availability, team productivity, patient summary, and encounter summary. The clinic availability view summarizes clinic capacity, no shows, overbookings, and percent utilization. Relative value unit- based productivity is summarized in the team productivity view. The patient summary view presents aggregated data for veterans served by NTO, including geographic distribution, with patient-level drill down displaying demographics, cancer characteristics, and treatment history. Lastly, the encounter view displays utilization trends by modality, while accurately accounting for the hub and spoke clinical model.

Conclusions

An informatics architecture and frontend information display that is capable of synthesizing EHR data into a consumable format has been pivotal in obtaining accurate and timely insight into the demand and capacity of services provided by NTO.

Background

Since inception, the Veterans Affairs (VA) National TeleOncology (NTO) service has monitored clinical operations through data tools produced by the Veterans Health Administration Support Service Center (VSSC). Unfortunately, pertinent data are spread across multiple reports, making it difficult to continually harmonize needed information. Further, the VSSC does not account for NTO’s hub and spoke clinical model, leading to inaccuracies when attempting to analyze unique encounters. To address these challenges, NTO partnered with the VA Salt Lake City Health Care System Informatics, Decision-Enhancement, and Analytic Sciences Center (IDEAS) to develop an informatics architecture and frontend NTO Clinical Operations Dashboard (NCOD). Here, we summarize our dashboard development process and the finalized key reporting components of the NCOD.

Methods

The VA Corporate Data Warehouse (CDW) serves as the primary data source for the NCOD. SQL Server Integration Services was used to build the backend data architecture. Data from the CDW were isolated into a staging data mart for reporting purposes using an extract, transform, load (ETL) approach. The frontend user interface was developed using Power BI. We used a participatory approach1 in determining reporting requirements. Stakeholders included the IDEAS dashboard development team and potential end users from NTO, including leadership, program managers, support assistants, and telehealth coordinators.

Results

The NCOD ETL is scheduled to refresh the data nightly to provide end users with a near real-time experience. The NCOD is comprised of the following four data views: clinic availability, team productivity, patient summary, and encounter summary. The clinic availability view summarizes clinic capacity, no shows, overbookings, and percent utilization. Relative value unit- based productivity is summarized in the team productivity view. The patient summary view presents aggregated data for veterans served by NTO, including geographic distribution, with patient-level drill down displaying demographics, cancer characteristics, and treatment history. Lastly, the encounter view displays utilization trends by modality, while accurately accounting for the hub and spoke clinical model.

Conclusions

An informatics architecture and frontend information display that is capable of synthesizing EHR data into a consumable format has been pivotal in obtaining accurate and timely insight into the demand and capacity of services provided by NTO.

Introduction

Hypereosinophilia can be seen in many medical conditions, including myeloproliferative disorders, and can lead to serious complications if untreated. Sweet syndrome is a rare and painful cutaneous inflammatory condition that has been linked to underlying malignancies.

Case Presentation

A 72-year-old male presented with 6-month history of painful maculopapular rash, night sweats, fever, and weight loss. He was treated with antibiotics and steroids with no improvement. A skin biopsy demonstrated neutrophilic dermatosis consistent with sweet syndrome. Laboratory studies a showed hemoglobin 7.1g/dl, WBC 12.9x103/uL, 30% eosinophils, absolute eosinophil count 3x109/L, and normal platelets. Infectious and immunological work up was negative. CT scan revealed splenomegaly. Bone marrow biopsy showed 100% hypercellularity, trilineage atypia, eosinophils 43% (normal, 1-5%) and 3-4% blasts positive for CD34 and CD117. FISH studies detected loss of PDGFRB signal, cytogenetics revealed a complex karyotype. He was diagnosed with a high-risk (based on IPSS-R) MDS/MPN cross-over with peripheral eosinophilia and is planned to undergo HSCT.

Discussion

Hematologic malignancies are associated with several paraneoplastic syndromes including sweet syndrome, also known as acute febrile neutrophilic dermatosis. The literature describes sweet syndrome occurring mostly with AML but can also be seen with other malignancies like MDS and solid tumor. The distinction between sweet syndrome and infectious or immune-mediated rash can be challenging as it requires histopathologic evaluation and is usually mistreated. Hypereosinophilia is defined as persistent eosinophil count of at least 1.5x109/L. It can be idiopathic or associated with allergic, rheumatologic, infectious, or neoplastic conditions. Clonal hypereosinophilia is most frequently associated with chronic myeloid neoplasms such as myeloproliferative neoplasm (MPN) or overlapping MDS/MPN, and more less frequently with AML. Hypereosinophilia related to hematological malignancies has been linked to gene rearrangements involving PDGFRA, PDGFRB, FGFR1, and JAK2. Patients with documented rearrangements or mutations in PDGFRB are treated with imatinib, which is a potent kinase inhibitor. However, patients with high-risk MDS/MPN with associated eosinophilia are typically treated as MDS and should undergo allogenic HSCT if eligible.

Conclusions

Both hypereosinophlia and sweet syndrome have been linked to myeloid neoplasms. Early recognition of either phenomenon as a paraneoplastic syndrome is important for early diagnosis and treatment.

Introduction

Hypereosinophilia can be seen in many medical conditions, including myeloproliferative disorders, and can lead to serious complications if untreated. Sweet syndrome is a rare and painful cutaneous inflammatory condition that has been linked to underlying malignancies.

Case Presentation

A 72-year-old male presented with 6-month history of painful maculopapular rash, night sweats, fever, and weight loss. He was treated with antibiotics and steroids with no improvement. A skin biopsy demonstrated neutrophilic dermatosis consistent with sweet syndrome. Laboratory studies a showed hemoglobin 7.1g/dl, WBC 12.9x103/uL, 30% eosinophils, absolute eosinophil count 3x109/L, and normal platelets. Infectious and immunological work up was negative. CT scan revealed splenomegaly. Bone marrow biopsy showed 100% hypercellularity, trilineage atypia, eosinophils 43% (normal, 1-5%) and 3-4% blasts positive for CD34 and CD117. FISH studies detected loss of PDGFRB signal, cytogenetics revealed a complex karyotype. He was diagnosed with a high-risk (based on IPSS-R) MDS/MPN cross-over with peripheral eosinophilia and is planned to undergo HSCT.

Discussion

Hematologic malignancies are associated with several paraneoplastic syndromes including sweet syndrome, also known as acute febrile neutrophilic dermatosis. The literature describes sweet syndrome occurring mostly with AML but can also be seen with other malignancies like MDS and solid tumor. The distinction between sweet syndrome and infectious or immune-mediated rash can be challenging as it requires histopathologic evaluation and is usually mistreated. Hypereosinophilia is defined as persistent eosinophil count of at least 1.5x109/L. It can be idiopathic or associated with allergic, rheumatologic, infectious, or neoplastic conditions. Clonal hypereosinophilia is most frequently associated with chronic myeloid neoplasms such as myeloproliferative neoplasm (MPN) or overlapping MDS/MPN, and more less frequently with AML. Hypereosinophilia related to hematological malignancies has been linked to gene rearrangements involving PDGFRA, PDGFRB, FGFR1, and JAK2. Patients with documented rearrangements or mutations in PDGFRB are treated with imatinib, which is a potent kinase inhibitor. However, patients with high-risk MDS/MPN with associated eosinophilia are typically treated as MDS and should undergo allogenic HSCT if eligible.

Conclusions

Both hypereosinophlia and sweet syndrome have been linked to myeloid neoplasms. Early recognition of either phenomenon as a paraneoplastic syndrome is important for early diagnosis and treatment.

Introduction

Hypereosinophilia can be seen in many medical conditions, including myeloproliferative disorders, and can lead to serious complications if untreated. Sweet syndrome is a rare and painful cutaneous inflammatory condition that has been linked to underlying malignancies.

Case Presentation

A 72-year-old male presented with 6-month history of painful maculopapular rash, night sweats, fever, and weight loss. He was treated with antibiotics and steroids with no improvement. A skin biopsy demonstrated neutrophilic dermatosis consistent with sweet syndrome. Laboratory studies a showed hemoglobin 7.1g/dl, WBC 12.9x103/uL, 30% eosinophils, absolute eosinophil count 3x109/L, and normal platelets. Infectious and immunological work up was negative. CT scan revealed splenomegaly. Bone marrow biopsy showed 100% hypercellularity, trilineage atypia, eosinophils 43% (normal, 1-5%) and 3-4% blasts positive for CD34 and CD117. FISH studies detected loss of PDGFRB signal, cytogenetics revealed a complex karyotype. He was diagnosed with a high-risk (based on IPSS-R) MDS/MPN cross-over with peripheral eosinophilia and is planned to undergo HSCT.

Discussion

Hematologic malignancies are associated with several paraneoplastic syndromes including sweet syndrome, also known as acute febrile neutrophilic dermatosis. The literature describes sweet syndrome occurring mostly with AML but can also be seen with other malignancies like MDS and solid tumor. The distinction between sweet syndrome and infectious or immune-mediated rash can be challenging as it requires histopathologic evaluation and is usually mistreated. Hypereosinophilia is defined as persistent eosinophil count of at least 1.5x109/L. It can be idiopathic or associated with allergic, rheumatologic, infectious, or neoplastic conditions. Clonal hypereosinophilia is most frequently associated with chronic myeloid neoplasms such as myeloproliferative neoplasm (MPN) or overlapping MDS/MPN, and more less frequently with AML. Hypereosinophilia related to hematological malignancies has been linked to gene rearrangements involving PDGFRA, PDGFRB, FGFR1, and JAK2. Patients with documented rearrangements or mutations in PDGFRB are treated with imatinib, which is a potent kinase inhibitor. However, patients with high-risk MDS/MPN with associated eosinophilia are typically treated as MDS and should undergo allogenic HSCT if eligible.

Conclusions

Both hypereosinophlia and sweet syndrome have been linked to myeloid neoplasms. Early recognition of either phenomenon as a paraneoplastic syndrome is important for early diagnosis and treatment.

Background

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors with worse prognosis if arising from the small bowel. Surgery remains the mainstay of treatment for patients with resectable tumors. Imatinib has become the standard treatment in cKIT-positive GISTs with significant morbidity in neoadjuvant, adjuvant, and palliative settings. There are limited data on efficacy and safety of imatinib in dialysis patients, and chemotherapy dosing is challenging in dialysis patients with multiple comorbidities.

Presentation

A 68-year-old male with a history of orthotopic heart transplantation on sirolimus with prednisone, cardiac allograft vasculopathy, plus ESRD on peritoneal dialysis (PD), presented with lower abdominal pain and fever. Abdominal imaging revealed a right lower quadrant (RLQ) mass with concern for bowel perforation.

Diagnosis and Treatment

The patient underwent exploratory laparoscopy with small bowel resection, excision of the mesenteric small bowel mass, drainage and washout of intraabdominal abscess, removal of PD catheter, and transition to hemodialysis. Pathology revealed a 14.5-cm high-grade GIST with mixed spindle and epithelioid types involving the ileal wall and mesentery, consistent with pT4 primary tumor and stage IIIB disease. Molecular testing was positive for c-KIT and DOG-1 mutations.

After a prolonged recovery, repeat abdominal imaging demonstrated metastatic liver disease and a new RLQ lesion. The patient was started on palliative imatinib 100 mg daily with subsequent increase to 200 mg daily. He was monitored closely for toxicities but reported only mild nausea controlled with ondansetron. Hemodialysis was continued 3 times per week. Follow up scans 3 months later showed improvement in RLQ mass and hepatic lesions. The patient remains on the current dose 15 months after the diagnosis.

Conclusion

To our knowledge, this is the first case of a small intestinal GIST in a heart transplant recipient treated with dose-reduced imatinib with concurrent dialysis and immunosuppression. Treatment decision-making was complex given concern for cardiotoxicity with pre-existing cardiac disease and drug-drug interactions with immunosuppressive agents. While some literature suggests standard dose imatinib with dialysis, no large-scale studies evaluated pharmacokinetics of imatinib with creatinine clearance < 20 mL/min. There is a need for further studies to determine dosing strategies for such patients.

Background

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors with worse prognosis if arising from the small bowel. Surgery remains the mainstay of treatment for patients with resectable tumors. Imatinib has become the standard treatment in cKIT-positive GISTs with significant morbidity in neoadjuvant, adjuvant, and palliative settings. There are limited data on efficacy and safety of imatinib in dialysis patients, and chemotherapy dosing is challenging in dialysis patients with multiple comorbidities.

Presentation

A 68-year-old male with a history of orthotopic heart transplantation on sirolimus with prednisone, cardiac allograft vasculopathy, plus ESRD on peritoneal dialysis (PD), presented with lower abdominal pain and fever. Abdominal imaging revealed a right lower quadrant (RLQ) mass with concern for bowel perforation.

Diagnosis and Treatment

The patient underwent exploratory laparoscopy with small bowel resection, excision of the mesenteric small bowel mass, drainage and washout of intraabdominal abscess, removal of PD catheter, and transition to hemodialysis. Pathology revealed a 14.5-cm high-grade GIST with mixed spindle and epithelioid types involving the ileal wall and mesentery, consistent with pT4 primary tumor and stage IIIB disease. Molecular testing was positive for c-KIT and DOG-1 mutations.

After a prolonged recovery, repeat abdominal imaging demonstrated metastatic liver disease and a new RLQ lesion. The patient was started on palliative imatinib 100 mg daily with subsequent increase to 200 mg daily. He was monitored closely for toxicities but reported only mild nausea controlled with ondansetron. Hemodialysis was continued 3 times per week. Follow up scans 3 months later showed improvement in RLQ mass and hepatic lesions. The patient remains on the current dose 15 months after the diagnosis.

Conclusion

To our knowledge, this is the first case of a small intestinal GIST in a heart transplant recipient treated with dose-reduced imatinib with concurrent dialysis and immunosuppression. Treatment decision-making was complex given concern for cardiotoxicity with pre-existing cardiac disease and drug-drug interactions with immunosuppressive agents. While some literature suggests standard dose imatinib with dialysis, no large-scale studies evaluated pharmacokinetics of imatinib with creatinine clearance < 20 mL/min. There is a need for further studies to determine dosing strategies for such patients.

Background

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal tumors with worse prognosis if arising from the small bowel. Surgery remains the mainstay of treatment for patients with resectable tumors. Imatinib has become the standard treatment in cKIT-positive GISTs with significant morbidity in neoadjuvant, adjuvant, and palliative settings. There are limited data on efficacy and safety of imatinib in dialysis patients, and chemotherapy dosing is challenging in dialysis patients with multiple comorbidities.

Presentation

A 68-year-old male with a history of orthotopic heart transplantation on sirolimus with prednisone, cardiac allograft vasculopathy, plus ESRD on peritoneal dialysis (PD), presented with lower abdominal pain and fever. Abdominal imaging revealed a right lower quadrant (RLQ) mass with concern for bowel perforation.

Diagnosis and Treatment

The patient underwent exploratory laparoscopy with small bowel resection, excision of the mesenteric small bowel mass, drainage and washout of intraabdominal abscess, removal of PD catheter, and transition to hemodialysis. Pathology revealed a 14.5-cm high-grade GIST with mixed spindle and epithelioid types involving the ileal wall and mesentery, consistent with pT4 primary tumor and stage IIIB disease. Molecular testing was positive for c-KIT and DOG-1 mutations.

After a prolonged recovery, repeat abdominal imaging demonstrated metastatic liver disease and a new RLQ lesion. The patient was started on palliative imatinib 100 mg daily with subsequent increase to 200 mg daily. He was monitored closely for toxicities but reported only mild nausea controlled with ondansetron. Hemodialysis was continued 3 times per week. Follow up scans 3 months later showed improvement in RLQ mass and hepatic lesions. The patient remains on the current dose 15 months after the diagnosis.

Conclusion

To our knowledge, this is the first case of a small intestinal GIST in a heart transplant recipient treated with dose-reduced imatinib with concurrent dialysis and immunosuppression. Treatment decision-making was complex given concern for cardiotoxicity with pre-existing cardiac disease and drug-drug interactions with immunosuppressive agents. While some literature suggests standard dose imatinib with dialysis, no large-scale studies evaluated pharmacokinetics of imatinib with creatinine clearance < 20 mL/min. There is a need for further studies to determine dosing strategies for such patients.

Objectives

US Department of Veterans Affairs (VA) patients with genetics referrals are 1.5 times more likely to have multiple cancer screening and preventive procedures if they completed their genetic consultations, but only when completed under a VA traditional model staffed by a team of clinical geneticists and genetic counselors versus a VA nontraditional, centralized telehealth model staffed by genetic counselors working independently. We sought to understand the reasons for these differences in cancer screening and prevention uptake.

Methods

We reviewed randomly selected medical records of patients with cancer genetics referrals stratified by model (142 records from each model). We conducted semi-structured interviews with 15 genetics providers and 36 referring clinicians from 13 VA facilities using purposive sampling. We analyzed annotated medical records and interview transcripts using a rapid assessment process. We characterized annotations as personalized recommendations (eg, begin colonoscopy at age 30 then every 1-2 years), options (eg, consider bilateral mastectomy), and generic messages (eg, refer to guidelines or another provider).

Results

Cancer screening or prevention was documented in 80 traditional-model records (141 annotations) and 106 nontraditional-model records (143 annotations). Personalized recommendations comprised 69% (97/141) of annotations within traditional-model records and 30% (43/143) within nontraditional-model records. Generic messages comprised 17% (24/141) of annotations in traditional-model records and 51% (73/143) in nontraditional-model records. From interview data, referring clinicians expected a broad range of services from genetics providers, including management and screening recommendations, and stated their role was to follow through on recommendations made. Under the traditional model, geneticists formulated recommendations documented by genetic counselors. Under the nontraditional model, scope of practice limited how genetic counselors addressed cancer screening and prevention.

Conclusions/Impacts

Personalized recommendations were typical of traditional-model records, whereas nontraditional-model records usually had generic messages. Compared with the nontraditional model, the traditional model was more patient-centered and better meets expectations of referring clinicians, which might explain in part the differences in patient uptake of cancer screening and preventive procedures.

As the demand for genetic services grows, the VA should promote team-based care under the traditional model for a more patient-centered, coordinated, and effective system of care for precision oncology.

Objectives

US Department of Veterans Affairs (VA) patients with genetics referrals are 1.5 times more likely to have multiple cancer screening and preventive procedures if they completed their genetic consultations, but only when completed under a VA traditional model staffed by a team of clinical geneticists and genetic counselors versus a VA nontraditional, centralized telehealth model staffed by genetic counselors working independently. We sought to understand the reasons for these differences in cancer screening and prevention uptake.

Methods

We reviewed randomly selected medical records of patients with cancer genetics referrals stratified by model (142 records from each model). We conducted semi-structured interviews with 15 genetics providers and 36 referring clinicians from 13 VA facilities using purposive sampling. We analyzed annotated medical records and interview transcripts using a rapid assessment process. We characterized annotations as personalized recommendations (eg, begin colonoscopy at age 30 then every 1-2 years), options (eg, consider bilateral mastectomy), and generic messages (eg, refer to guidelines or another provider).

Results

Cancer screening or prevention was documented in 80 traditional-model records (141 annotations) and 106 nontraditional-model records (143 annotations). Personalized recommendations comprised 69% (97/141) of annotations within traditional-model records and 30% (43/143) within nontraditional-model records. Generic messages comprised 17% (24/141) of annotations in traditional-model records and 51% (73/143) in nontraditional-model records. From interview data, referring clinicians expected a broad range of services from genetics providers, including management and screening recommendations, and stated their role was to follow through on recommendations made. Under the traditional model, geneticists formulated recommendations documented by genetic counselors. Under the nontraditional model, scope of practice limited how genetic counselors addressed cancer screening and prevention.

Conclusions/Impacts

Personalized recommendations were typical of traditional-model records, whereas nontraditional-model records usually had generic messages. Compared with the nontraditional model, the traditional model was more patient-centered and better meets expectations of referring clinicians, which might explain in part the differences in patient uptake of cancer screening and preventive procedures.

As the demand for genetic services grows, the VA should promote team-based care under the traditional model for a more patient-centered, coordinated, and effective system of care for precision oncology.

Objectives

US Department of Veterans Affairs (VA) patients with genetics referrals are 1.5 times more likely to have multiple cancer screening and preventive procedures if they completed their genetic consultations, but only when completed under a VA traditional model staffed by a team of clinical geneticists and genetic counselors versus a VA nontraditional, centralized telehealth model staffed by genetic counselors working independently. We sought to understand the reasons for these differences in cancer screening and prevention uptake.

Methods

We reviewed randomly selected medical records of patients with cancer genetics referrals stratified by model (142 records from each model). We conducted semi-structured interviews with 15 genetics providers and 36 referring clinicians from 13 VA facilities using purposive sampling. We analyzed annotated medical records and interview transcripts using a rapid assessment process. We characterized annotations as personalized recommendations (eg, begin colonoscopy at age 30 then every 1-2 years), options (eg, consider bilateral mastectomy), and generic messages (eg, refer to guidelines or another provider).

Results

Cancer screening or prevention was documented in 80 traditional-model records (141 annotations) and 106 nontraditional-model records (143 annotations). Personalized recommendations comprised 69% (97/141) of annotations within traditional-model records and 30% (43/143) within nontraditional-model records. Generic messages comprised 17% (24/141) of annotations in traditional-model records and 51% (73/143) in nontraditional-model records. From interview data, referring clinicians expected a broad range of services from genetics providers, including management and screening recommendations, and stated their role was to follow through on recommendations made. Under the traditional model, geneticists formulated recommendations documented by genetic counselors. Under the nontraditional model, scope of practice limited how genetic counselors addressed cancer screening and prevention.

Conclusions/Impacts

Personalized recommendations were typical of traditional-model records, whereas nontraditional-model records usually had generic messages. Compared with the nontraditional model, the traditional model was more patient-centered and better meets expectations of referring clinicians, which might explain in part the differences in patient uptake of cancer screening and preventive procedures.

As the demand for genetic services grows, the VA should promote team-based care under the traditional model for a more patient-centered, coordinated, and effective system of care for precision oncology.