Cancer

Federal Health Care Data Trends (click to view the digital edition) is a special supplement to Federal Practitioner, highlighting the latest research and study outcomes related to the health of veteran and active-duty populations.

In this issue: 

• Limb Loss and Prostheses
• Neurology
  • Traumatic Brain Injury
  • Alzheimer's and Dementia
  • Amyotrophic Lateral Sclerosis (ALS)
  • Parkinson's Disease
  • Migraine and Headache
• Cardiology
• Mental Health
  • Depression
  • Opioid Use Disorder
  • PTSD and Psychedelic Treatments
  • Homelessness
  • Eating Disorders
• Diabetes
• Rheumatoid Arthritis
• Respiratory illnesses
• Women's Health
  • Access to Care
  • Infertility
  • Pregnancy
• HPV and Related Cancers

Federal Health Care Data Trends (click to view the digital edition) is a special supplement to Federal Practitioner, highlighting the latest research and study outcomes related to the health of veteran and active-duty populations.

In this issue: 

• Limb Loss and Prostheses
• Neurology
  • Traumatic Brain Injury
  • Alzheimer's and Dementia
  • Amyotrophic Lateral Sclerosis (ALS)
  • Parkinson's Disease
  • Migraine and Headache
• Cardiology
• Mental Health
  • Depression
  • Opioid Use Disorder
  • PTSD and Psychedelic Treatments
  • Homelessness
  • Eating Disorders
• Diabetes
• Rheumatoid Arthritis
• Respiratory illnesses
• Women's Health
  • Access to Care
  • Infertility
  • Pregnancy
• HPV and Related Cancers

Federal Health Care Data Trends (click to view the digital edition) is a special supplement to Federal Practitioner, highlighting the latest research and study outcomes related to the health of veteran and active-duty populations.

In this issue: 

• Limb Loss and Prostheses
• Neurology
  • Traumatic Brain Injury
  • Alzheimer's and Dementia
  • Amyotrophic Lateral Sclerosis (ALS)
  • Parkinson's Disease
  • Migraine and Headache
• Cardiology
• Mental Health
  • Depression
  • Opioid Use Disorder
  • PTSD and Psychedelic Treatments
  • Homelessness
  • Eating Disorders
• Diabetes
• Rheumatoid Arthritis
• Respiratory illnesses
• Women's Health
  • Access to Care
  • Infertility
  • Pregnancy
• HPV and Related Cancers

Click to read more from Federal Health Care Data Trends 2023.

Click to read more from Federal Health Care Data Trends 2023.

Click to read more from Federal Health Care Data Trends 2023.

CHICAGO – The US Department of Veterans Affairs (VA) top medical officer told the Association of VA Hematology/Oncology (AVAHO) oncology members that they are at the forefront of the agency’s push to expand care for veterans who may have been injured by exposure to hazardous materials on the job.

“All of you are playing a critical role in implementing the PACT Act, the signature legislative achievement of the Biden administration,” said Shereef Elnahal, MD, MBA, the VA Under Secretary for Health, in a keynote address at the 2023 annual meeting of AVAHO. “But more importantly, if we do our jobs right, it could be the largest expansion of veterans’ benefits in the history of this country. That requires us to have the capacity to deliver care to so many more individuals.”

The VA has provided more than 4.1 million free toxic exposure screenings to veterans since President Biden signed the PACT Act (The Sergeant First Class Heath Robinson Honoring our Promise to Address Comprehensive Toxics Act) in August 2022. The legislation prioritizes claims for cancer, terminal illnesses, and homelessness, and the White House says it has allowed the Veterans Health Administration and the Veterans Benefits Administration to grow at the fastest rates in 2 decades. 

“Almost every type of solid tumor is now considered a presumptive condition associated with burden of exposure to veterans deployed anywhere in Central Command, either in the Persian Gulf War or the post-9/11 conflicts,” said Under Secretary Elnahal, who was confirmed in his job by the Senate in July 2022. 

Implementing the PACT Act “requires all of us to make investments and further strengthen our system’s care for oncology,” he said. It is also crucial, to reduce “leakage into the community,” referring to veterans who leave the VA for private care. “I know for a fact that the care that veterans get when they have oncology services available in our direct-care system within VA is better. That's not a contention. That is proven by different peer-reviewed studies over the years. And I think that comparison is only intensifying when it comes to how much better evidence-based care our veterans receive at the hands of all of you across the country.”

Elnahal highlighted the development of a “2-way” cancer registry that will allow the National Institute and the VA to exchange cancer diagnosis and treatment data with state registries. “This will give the VA access to critical data in a complete way—to what veterans have experienced, especially veterans who are getting parts of their care in one place and parts of their care in a different place.”

On the data front, he also noted that “the PACT Act also requires us to research the future and determine the next set of presumptive conditions that are related to the hazards of serving our country. It requires that we have robust data sets to be able to gain those insights.”
More globally, Elnahal said the VA can play a crucial role in the Cancer Moonshot Program: “We can win the race, and VA can contribute asymmetrically to that race, to make cancer a chronic condition.”

He highlighted efforts within the VA to battle cancer such as programs to reduce disparities, boost cancer screening, treat rural veterans via a national teleoncology service, and implement the Close to Me program to bring infusion services to veterans in isolated regions.

But Elnahal’s presentation was not entirely rosy. He warned that 40% of veterans are being served outside the VA. “That's sort of a rule-of-thumb threshold when you start looking more like a payer than a provider.”

He also noted that while the VA hired 54,000 people in just the past year—6.2% growth—it takes a long time to bring workers on board. “That’s why I'm holding every single leader in our system accountable for reducing onboarding times by at least a month,” he said. The AVAHO audience enthusiastically applauded.

CHICAGO – The US Department of Veterans Affairs (VA) top medical officer told the Association of VA Hematology/Oncology (AVAHO) oncology members that they are at the forefront of the agency’s push to expand care for veterans who may have been injured by exposure to hazardous materials on the job.

“All of you are playing a critical role in implementing the PACT Act, the signature legislative achievement of the Biden administration,” said Shereef Elnahal, MD, MBA, the VA Under Secretary for Health, in a keynote address at the 2023 annual meeting of AVAHO. “But more importantly, if we do our jobs right, it could be the largest expansion of veterans’ benefits in the history of this country. That requires us to have the capacity to deliver care to so many more individuals.”

The VA has provided more than 4.1 million free toxic exposure screenings to veterans since President Biden signed the PACT Act (The Sergeant First Class Heath Robinson Honoring our Promise to Address Comprehensive Toxics Act) in August 2022. The legislation prioritizes claims for cancer, terminal illnesses, and homelessness, and the White House says it has allowed the Veterans Health Administration and the Veterans Benefits Administration to grow at the fastest rates in 2 decades. 

“Almost every type of solid tumor is now considered a presumptive condition associated with burden of exposure to veterans deployed anywhere in Central Command, either in the Persian Gulf War or the post-9/11 conflicts,” said Under Secretary Elnahal, who was confirmed in his job by the Senate in July 2022. 

Implementing the PACT Act “requires all of us to make investments and further strengthen our system’s care for oncology,” he said. It is also crucial, to reduce “leakage into the community,” referring to veterans who leave the VA for private care. “I know for a fact that the care that veterans get when they have oncology services available in our direct-care system within VA is better. That's not a contention. That is proven by different peer-reviewed studies over the years. And I think that comparison is only intensifying when it comes to how much better evidence-based care our veterans receive at the hands of all of you across the country.”

Elnahal highlighted the development of a “2-way” cancer registry that will allow the National Institute and the VA to exchange cancer diagnosis and treatment data with state registries. “This will give the VA access to critical data in a complete way—to what veterans have experienced, especially veterans who are getting parts of their care in one place and parts of their care in a different place.”

On the data front, he also noted that “the PACT Act also requires us to research the future and determine the next set of presumptive conditions that are related to the hazards of serving our country. It requires that we have robust data sets to be able to gain those insights.”
More globally, Elnahal said the VA can play a crucial role in the Cancer Moonshot Program: “We can win the race, and VA can contribute asymmetrically to that race, to make cancer a chronic condition.”

He highlighted efforts within the VA to battle cancer such as programs to reduce disparities, boost cancer screening, treat rural veterans via a national teleoncology service, and implement the Close to Me program to bring infusion services to veterans in isolated regions.

But Elnahal’s presentation was not entirely rosy. He warned that 40% of veterans are being served outside the VA. “That's sort of a rule-of-thumb threshold when you start looking more like a payer than a provider.”

He also noted that while the VA hired 54,000 people in just the past year—6.2% growth—it takes a long time to bring workers on board. “That’s why I'm holding every single leader in our system accountable for reducing onboarding times by at least a month,” he said. The AVAHO audience enthusiastically applauded.

CHICAGO – The US Department of Veterans Affairs (VA) top medical officer told the Association of VA Hematology/Oncology (AVAHO) oncology members that they are at the forefront of the agency’s push to expand care for veterans who may have been injured by exposure to hazardous materials on the job.

“All of you are playing a critical role in implementing the PACT Act, the signature legislative achievement of the Biden administration,” said Shereef Elnahal, MD, MBA, the VA Under Secretary for Health, in a keynote address at the 2023 annual meeting of AVAHO. “But more importantly, if we do our jobs right, it could be the largest expansion of veterans’ benefits in the history of this country. That requires us to have the capacity to deliver care to so many more individuals.”

The VA has provided more than 4.1 million free toxic exposure screenings to veterans since President Biden signed the PACT Act (The Sergeant First Class Heath Robinson Honoring our Promise to Address Comprehensive Toxics Act) in August 2022. The legislation prioritizes claims for cancer, terminal illnesses, and homelessness, and the White House says it has allowed the Veterans Health Administration and the Veterans Benefits Administration to grow at the fastest rates in 2 decades. 

“Almost every type of solid tumor is now considered a presumptive condition associated with burden of exposure to veterans deployed anywhere in Central Command, either in the Persian Gulf War or the post-9/11 conflicts,” said Under Secretary Elnahal, who was confirmed in his job by the Senate in July 2022. 

Implementing the PACT Act “requires all of us to make investments and further strengthen our system’s care for oncology,” he said. It is also crucial, to reduce “leakage into the community,” referring to veterans who leave the VA for private care. “I know for a fact that the care that veterans get when they have oncology services available in our direct-care system within VA is better. That's not a contention. That is proven by different peer-reviewed studies over the years. And I think that comparison is only intensifying when it comes to how much better evidence-based care our veterans receive at the hands of all of you across the country.”

Elnahal highlighted the development of a “2-way” cancer registry that will allow the National Institute and the VA to exchange cancer diagnosis and treatment data with state registries. “This will give the VA access to critical data in a complete way—to what veterans have experienced, especially veterans who are getting parts of their care in one place and parts of their care in a different place.”

On the data front, he also noted that “the PACT Act also requires us to research the future and determine the next set of presumptive conditions that are related to the hazards of serving our country. It requires that we have robust data sets to be able to gain those insights.”
More globally, Elnahal said the VA can play a crucial role in the Cancer Moonshot Program: “We can win the race, and VA can contribute asymmetrically to that race, to make cancer a chronic condition.”

He highlighted efforts within the VA to battle cancer such as programs to reduce disparities, boost cancer screening, treat rural veterans via a national teleoncology service, and implement the Close to Me program to bring infusion services to veterans in isolated regions.

But Elnahal’s presentation was not entirely rosy. He warned that 40% of veterans are being served outside the VA. “That's sort of a rule-of-thumb threshold when you start looking more like a payer than a provider.”

He also noted that while the VA hired 54,000 people in just the past year—6.2% growth—it takes a long time to bring workers on board. “That’s why I'm holding every single leader in our system accountable for reducing onboarding times by at least a month,” he said. The AVAHO audience enthusiastically applauded.

At a glance, “health literacy” sounds like it has something specifically to do with the ability to read. Mary Laudon Thomas, MS, CNS, AOCN, a former president of Association of VA Hematology/Oncology, knows better.

“It’s not the same as reading level, and it’s not the same as educational level,” Thomas told Federal Practitioner. “Even educated people can think men can’t get breast cancer or misunderstand how to properly take their medications.”

Instead, health literacy is a broader topic: Do patients understand what’s going on when they get medical care? Can they use the information they get to make informed decisions about their health? Low health literacy is associated with lower use of preventative care of poorer adherence, poorer ability to navigate the health system and contributes to social inequities. In cancer care, low health literacy is associated with lower levels of screening, longer lag times in symptom identification, impairments in risk perception, fewer questions, lower perceived quality of life, and less follow-up.

Thomas and colleagues explored strategies to improve health literacy in cancer care during a half-day session on September 28th, kicking off the AVAHO 2023 annual meeting in Chicago. 

There are countless examples of patients who fail to understand aspects of their care, said Thomas, a retired clinical nurse specialist in hematology at California’s VA Palo Alto Health Care System who now serves as cochair of the AVAHO education committee. A patient may not realize that high blood pressure and hypertension are the same thing, for instance, or not understand that they need to go to the radiology department for a computed tomography.

“That’s our problem,” Thomas said. “We’re so fluent in our medical-speak that we forget we’re speaking a foreign language to other people.”

The goal of the AVAHO 2023 workshop is to “help people develop awareness of the scope of the problem and give them tools they can use to simplify how they speak to patients, teach patients and inform patients,” Thomas said.

In the first segment of the program, Angela Kumar, MPH, national program manager for Veterans Health Education and Information, discussed the VA organizational approach to health literacy. She noted that building a health-literate care organization aligns with the VA goal to be a high reliability organization. Veterans who have questions and concerns will need additional information throughout their cancer journey. The role for VA clinicians is to help answer veterans’ questions. “Rather than assume patients know what we are talking about, we have to make sure they understand,” Kumar explained. Institutional support will lead to better health outcomes and patient satisfaction throughout the system. VA is in the process of creating a patient centered learning program, Kumar noted. The program will be open to veterans, their families, caregivers, and provide training for VA health care professionals.

In the workshop’s 2 other sessions Janet Papadakos, PhD, MEd, a scientist at the University of Toronto’s Institute for Education Research, discussed the impact of health literacy on cancer treatment and outcomes and Fatemeh Youssefi, PhD, RN, OCN, director at large and committee member of the Oncology Nursing Society, discussed the roles of health literacy and patient education in empowering patients. Both speakers noted that patients with cancer are undergoing intense emotional stress, which can significantly impact their ability to understanding their treatment. Importantly, Papadakos explained, people can change and improve their health literacy, so clinicians have an opportunity to help influence and improve comprehension for their patient, by taking basic steps shown to improve health literacy.

“We know that in general, people with low health literacy report worse health, and they also have historically have poor outcomes,” Thomas said. Indeed, a 2021 systematic review of 66 papers found that “lower health literacy was associated with greater difficulties understanding and processing cancer related information, poorer quality of life and poorer experience of care.” Just 12% of US adults have proficient health literacy and one-third of adults have difficulty with common health tasks.

Papadakos and Youssefi provided some guidance for better communication with patients. Teach back, for example, is a tool to ensure patients understand topics when discussed. The key, Papadakos explained, is that it is not a test of the patient but rather a test of how well the information was communicated. Youssefi and Papadakos also emphasized the importance of using plain language. Clear and precise words that avoid technical terms avoid miscommunication and confusion. Finally, they urged clinicians to never assume health literacy and to approach all patients using clear language to ensure that they understand and can provide back the content covered.

Thomas said 3 more virtual sessions about health literacy will be offered over the coming year. Organizers will develop the specific topics after engaging in a discussion with attendees at the end of the AVAHO session. Meanwhile, advocates are developing a section of the AVAHO website that will be devoted to health literacy.

The workshop received support from Genentech.

At a glance, “health literacy” sounds like it has something specifically to do with the ability to read. Mary Laudon Thomas, MS, CNS, AOCN, a former president of Association of VA Hematology/Oncology, knows better.

“It’s not the same as reading level, and it’s not the same as educational level,” Thomas told Federal Practitioner. “Even educated people can think men can’t get breast cancer or misunderstand how to properly take their medications.”

Instead, health literacy is a broader topic: Do patients understand what’s going on when they get medical care? Can they use the information they get to make informed decisions about their health? Low health literacy is associated with lower use of preventative care of poorer adherence, poorer ability to navigate the health system and contributes to social inequities. In cancer care, low health literacy is associated with lower levels of screening, longer lag times in symptom identification, impairments in risk perception, fewer questions, lower perceived quality of life, and less follow-up.

Thomas and colleagues explored strategies to improve health literacy in cancer care during a half-day session on September 28th, kicking off the AVAHO 2023 annual meeting in Chicago. 

There are countless examples of patients who fail to understand aspects of their care, said Thomas, a retired clinical nurse specialist in hematology at California’s VA Palo Alto Health Care System who now serves as cochair of the AVAHO education committee. A patient may not realize that high blood pressure and hypertension are the same thing, for instance, or not understand that they need to go to the radiology department for a computed tomography.

“That’s our problem,” Thomas said. “We’re so fluent in our medical-speak that we forget we’re speaking a foreign language to other people.”

The goal of the AVAHO 2023 workshop is to “help people develop awareness of the scope of the problem and give them tools they can use to simplify how they speak to patients, teach patients and inform patients,” Thomas said.

In the first segment of the program, Angela Kumar, MPH, national program manager for Veterans Health Education and Information, discussed the VA organizational approach to health literacy. She noted that building a health-literate care organization aligns with the VA goal to be a high reliability organization. Veterans who have questions and concerns will need additional information throughout their cancer journey. The role for VA clinicians is to help answer veterans’ questions. “Rather than assume patients know what we are talking about, we have to make sure they understand,” Kumar explained. Institutional support will lead to better health outcomes and patient satisfaction throughout the system. VA is in the process of creating a patient centered learning program, Kumar noted. The program will be open to veterans, their families, caregivers, and provide training for VA health care professionals.

In the workshop’s 2 other sessions Janet Papadakos, PhD, MEd, a scientist at the University of Toronto’s Institute for Education Research, discussed the impact of health literacy on cancer treatment and outcomes and Fatemeh Youssefi, PhD, RN, OCN, director at large and committee member of the Oncology Nursing Society, discussed the roles of health literacy and patient education in empowering patients. Both speakers noted that patients with cancer are undergoing intense emotional stress, which can significantly impact their ability to understanding their treatment. Importantly, Papadakos explained, people can change and improve their health literacy, so clinicians have an opportunity to help influence and improve comprehension for their patient, by taking basic steps shown to improve health literacy.

“We know that in general, people with low health literacy report worse health, and they also have historically have poor outcomes,” Thomas said. Indeed, a 2021 systematic review of 66 papers found that “lower health literacy was associated with greater difficulties understanding and processing cancer related information, poorer quality of life and poorer experience of care.” Just 12% of US adults have proficient health literacy and one-third of adults have difficulty with common health tasks.

Papadakos and Youssefi provided some guidance for better communication with patients. Teach back, for example, is a tool to ensure patients understand topics when discussed. The key, Papadakos explained, is that it is not a test of the patient but rather a test of how well the information was communicated. Youssefi and Papadakos also emphasized the importance of using plain language. Clear and precise words that avoid technical terms avoid miscommunication and confusion. Finally, they urged clinicians to never assume health literacy and to approach all patients using clear language to ensure that they understand and can provide back the content covered.

Thomas said 3 more virtual sessions about health literacy will be offered over the coming year. Organizers will develop the specific topics after engaging in a discussion with attendees at the end of the AVAHO session. Meanwhile, advocates are developing a section of the AVAHO website that will be devoted to health literacy.

The workshop received support from Genentech.

At a glance, “health literacy” sounds like it has something specifically to do with the ability to read. Mary Laudon Thomas, MS, CNS, AOCN, a former president of Association of VA Hematology/Oncology, knows better.

“It’s not the same as reading level, and it’s not the same as educational level,” Thomas told Federal Practitioner. “Even educated people can think men can’t get breast cancer or misunderstand how to properly take their medications.”

Instead, health literacy is a broader topic: Do patients understand what’s going on when they get medical care? Can they use the information they get to make informed decisions about their health? Low health literacy is associated with lower use of preventative care of poorer adherence, poorer ability to navigate the health system and contributes to social inequities. In cancer care, low health literacy is associated with lower levels of screening, longer lag times in symptom identification, impairments in risk perception, fewer questions, lower perceived quality of life, and less follow-up.

Thomas and colleagues explored strategies to improve health literacy in cancer care during a half-day session on September 28th, kicking off the AVAHO 2023 annual meeting in Chicago. 

There are countless examples of patients who fail to understand aspects of their care, said Thomas, a retired clinical nurse specialist in hematology at California’s VA Palo Alto Health Care System who now serves as cochair of the AVAHO education committee. A patient may not realize that high blood pressure and hypertension are the same thing, for instance, or not understand that they need to go to the radiology department for a computed tomography.

“That’s our problem,” Thomas said. “We’re so fluent in our medical-speak that we forget we’re speaking a foreign language to other people.”

The goal of the AVAHO 2023 workshop is to “help people develop awareness of the scope of the problem and give them tools they can use to simplify how they speak to patients, teach patients and inform patients,” Thomas said.

In the first segment of the program, Angela Kumar, MPH, national program manager for Veterans Health Education and Information, discussed the VA organizational approach to health literacy. She noted that building a health-literate care organization aligns with the VA goal to be a high reliability organization. Veterans who have questions and concerns will need additional information throughout their cancer journey. The role for VA clinicians is to help answer veterans’ questions. “Rather than assume patients know what we are talking about, we have to make sure they understand,” Kumar explained. Institutional support will lead to better health outcomes and patient satisfaction throughout the system. VA is in the process of creating a patient centered learning program, Kumar noted. The program will be open to veterans, their families, caregivers, and provide training for VA health care professionals.

In the workshop’s 2 other sessions Janet Papadakos, PhD, MEd, a scientist at the University of Toronto’s Institute for Education Research, discussed the impact of health literacy on cancer treatment and outcomes and Fatemeh Youssefi, PhD, RN, OCN, director at large and committee member of the Oncology Nursing Society, discussed the roles of health literacy and patient education in empowering patients. Both speakers noted that patients with cancer are undergoing intense emotional stress, which can significantly impact their ability to understanding their treatment. Importantly, Papadakos explained, people can change and improve their health literacy, so clinicians have an opportunity to help influence and improve comprehension for their patient, by taking basic steps shown to improve health literacy.

“We know that in general, people with low health literacy report worse health, and they also have historically have poor outcomes,” Thomas said. Indeed, a 2021 systematic review of 66 papers found that “lower health literacy was associated with greater difficulties understanding and processing cancer related information, poorer quality of life and poorer experience of care.” Just 12% of US adults have proficient health literacy and one-third of adults have difficulty with common health tasks.

Papadakos and Youssefi provided some guidance for better communication with patients. Teach back, for example, is a tool to ensure patients understand topics when discussed. The key, Papadakos explained, is that it is not a test of the patient but rather a test of how well the information was communicated. Youssefi and Papadakos also emphasized the importance of using plain language. Clear and precise words that avoid technical terms avoid miscommunication and confusion. Finally, they urged clinicians to never assume health literacy and to approach all patients using clear language to ensure that they understand and can provide back the content covered.

Thomas said 3 more virtual sessions about health literacy will be offered over the coming year. Organizers will develop the specific topics after engaging in a discussion with attendees at the end of the AVAHO session. Meanwhile, advocates are developing a section of the AVAHO website that will be devoted to health literacy.

The workshop received support from Genentech.

INTRODUCTION

Urinary bladder is an extremely rare site of extrapulmonary small cell cancer (EPSCC). Unlike small cell lung cancer (SCLC), there is no clear guideline for prophylactic cranial irradiation (PCI) for EPSCC. In this case report and literature review, we discuss small cell cancer of urinary bladder (SCCUB) and the role of PCI in SCCUB.

CASE PRESENTATION

A 74-year-old male presented with gross hematuria and an unremarkable physical examination. CT showed 1.7 cm right anterolateral bladder wall thickening. Cystoscopy revealed a 2-3 cm high-grade bladder lesion. Pathology from transurethral resection of the tumor was consistent with T1N0M0 small cell carcinoma. MRI brain and FDG-PET showed no extravesical disease. Patient received four cycles of neoadjuvant carboplatin/etoposide per his preference as he wanted to protect his hearing due to his profession followed by radical cystoprostatectomy. Post-op pathology showed clear margins. We decided to forego PCI in favor of interval surveillance with MRI and follow- up images remain negative for distant metastases.

DISCUSSION

EPSCC accounts for 2.5-5% of all SCC, very rare in male genitourinary tract. Treatment approach is derived from SCLC, guided by extent of disease and patient’s functional status. Role of PCI in EPSCC has not been clearly described, and even less evidence is available for SCCUB. From a review of eleven studies in PubMed for the role of PCI in SCCUB or EPSCC, we found that SCCUB has lower incidence of brain metastases than SCLC. One study suggested that SCCUB arises from totipotent cells in the submucosa, unlike Kulchitsky cell origin of SCLC. This difference might explain the difference in their metastatic behavior. With this background, PCI is not routinely recommended for limited- stage SCCUB. There might still be a role for PCI in extensive SCCUB with high metastatic burden. More studies are needed to update the guidelines for the role of PCI for these tumors.

CONCLUSIONS

Per this literature review, PCI is not routinely recommended for SCCUB, likely due to different cells of origin compared to SCLC. Future studies should focus on characterizing differences in their metastatic behavior and updating guidelines for PCI for SCCUB.

INTRODUCTION

Urinary bladder is an extremely rare site of extrapulmonary small cell cancer (EPSCC). Unlike small cell lung cancer (SCLC), there is no clear guideline for prophylactic cranial irradiation (PCI) for EPSCC. In this case report and literature review, we discuss small cell cancer of urinary bladder (SCCUB) and the role of PCI in SCCUB.

CASE PRESENTATION

A 74-year-old male presented with gross hematuria and an unremarkable physical examination. CT showed 1.7 cm right anterolateral bladder wall thickening. Cystoscopy revealed a 2-3 cm high-grade bladder lesion. Pathology from transurethral resection of the tumor was consistent with T1N0M0 small cell carcinoma. MRI brain and FDG-PET showed no extravesical disease. Patient received four cycles of neoadjuvant carboplatin/etoposide per his preference as he wanted to protect his hearing due to his profession followed by radical cystoprostatectomy. Post-op pathology showed clear margins. We decided to forego PCI in favor of interval surveillance with MRI and follow- up images remain negative for distant metastases.

DISCUSSION

EPSCC accounts for 2.5-5% of all SCC, very rare in male genitourinary tract. Treatment approach is derived from SCLC, guided by extent of disease and patient’s functional status. Role of PCI in EPSCC has not been clearly described, and even less evidence is available for SCCUB. From a review of eleven studies in PubMed for the role of PCI in SCCUB or EPSCC, we found that SCCUB has lower incidence of brain metastases than SCLC. One study suggested that SCCUB arises from totipotent cells in the submucosa, unlike Kulchitsky cell origin of SCLC. This difference might explain the difference in their metastatic behavior. With this background, PCI is not routinely recommended for limited- stage SCCUB. There might still be a role for PCI in extensive SCCUB with high metastatic burden. More studies are needed to update the guidelines for the role of PCI for these tumors.

CONCLUSIONS

Per this literature review, PCI is not routinely recommended for SCCUB, likely due to different cells of origin compared to SCLC. Future studies should focus on characterizing differences in their metastatic behavior and updating guidelines for PCI for SCCUB.

INTRODUCTION

Urinary bladder is an extremely rare site of extrapulmonary small cell cancer (EPSCC). Unlike small cell lung cancer (SCLC), there is no clear guideline for prophylactic cranial irradiation (PCI) for EPSCC. In this case report and literature review, we discuss small cell cancer of urinary bladder (SCCUB) and the role of PCI in SCCUB.

CASE PRESENTATION

A 74-year-old male presented with gross hematuria and an unremarkable physical examination. CT showed 1.7 cm right anterolateral bladder wall thickening. Cystoscopy revealed a 2-3 cm high-grade bladder lesion. Pathology from transurethral resection of the tumor was consistent with T1N0M0 small cell carcinoma. MRI brain and FDG-PET showed no extravesical disease. Patient received four cycles of neoadjuvant carboplatin/etoposide per his preference as he wanted to protect his hearing due to his profession followed by radical cystoprostatectomy. Post-op pathology showed clear margins. We decided to forego PCI in favor of interval surveillance with MRI and follow- up images remain negative for distant metastases.

DISCUSSION

EPSCC accounts for 2.5-5% of all SCC, very rare in male genitourinary tract. Treatment approach is derived from SCLC, guided by extent of disease and patient’s functional status. Role of PCI in EPSCC has not been clearly described, and even less evidence is available for SCCUB. From a review of eleven studies in PubMed for the role of PCI in SCCUB or EPSCC, we found that SCCUB has lower incidence of brain metastases than SCLC. One study suggested that SCCUB arises from totipotent cells in the submucosa, unlike Kulchitsky cell origin of SCLC. This difference might explain the difference in their metastatic behavior. With this background, PCI is not routinely recommended for limited- stage SCCUB. There might still be a role for PCI in extensive SCCUB with high metastatic burden. More studies are needed to update the guidelines for the role of PCI for these tumors.

CONCLUSIONS

Per this literature review, PCI is not routinely recommended for SCCUB, likely due to different cells of origin compared to SCLC. Future studies should focus on characterizing differences in their metastatic behavior and updating guidelines for PCI for SCCUB.

INTRODUCTION

Lymphadenopathy in Chronic Lymphocytic Leukemia (CLL) is a very common feature. However, sudden increase in lymphadenopathy or other symptoms like weight loss should be evaluated for possible metastatic malignancy. We describe a CLL patient with diffuse mediastinal lymphadenopathy who was diagnosed with metastatic bladder cancer without a primary bladder tumor mass on imaging.

CASE DESCRIPTION

A 60-year-old man with a 60 pack-year smoking history, alcoholic cirrhosis, and a 5-year history of stage 1 CLL presented with 3 months of progressive shortness of breath; persistent cough; chills; hemoptysis; and a steady weight loss of 35 lbs. Notably, he had no bladder symptoms. Initial labs showed leukocytosis of 35.8k with a lymphocytic predominance. Screening low-dose chest CT was positive for diffuse mediastinal lymphadenopathy. Subsequent PET/CT revealed numerous hypermetabolic lymph nodes in the neck, mediastinum, left hilum, and right periaortic abdominal region. CT Chest, Abdomen, Pelvis revealed progressive lymphadenopathy as seen in prior imaging, stable pulmonary nodules up to 4 mm in size, and splenomegaly. No distant primary sites, including of the bladder, were identified. Mediastinal lymph node biopsy confirmed metastatic poorly differentiated carcinoma with immunohistochemical staining negative for p40, p63, CK20, TTF-1, Napsin A, CDX2, CA19- 9, Calretinin, and D2-40 and positive for CK7, GATA3, Ber-EP4, and Uroplakin, supporting bladder as primary origin. Urology deferred a cystoscopy given his lack of urinary symptoms and positive biopsy and was started on Carboplatin/Gemcitabine for his metastatic disease. He was ineligible for Cisplatin given his cirrhosis and hearing impairment.

DISCUSSION

In patients with CLL, new onset mediastinal lymphadenopathy is concerning for disease progression and possible transformation to a diffuse b-cell lymphoma. However, this symptom has a broad differential, including primary lung carcinomas, sarcomas, and metastatic disease. While our patient’s PET/CT and pan-CT failed to identify a distant primary site, maintaining a low clinical suspicion for metastatic disease and doing a thorough work-up was paramount. Only through immunohistochemical staining were we able to diagnosis this patient with urothelial carcinoma.

CONCLUSIONS

Biopsy with immunohistochemical staining and maintaining a low suspicion for worsening lymphadenopathy can identify unusually presenting urothelial carcinomas in CLL patients.

INTRODUCTION

Lymphadenopathy in Chronic Lymphocytic Leukemia (CLL) is a very common feature. However, sudden increase in lymphadenopathy or other symptoms like weight loss should be evaluated for possible metastatic malignancy. We describe a CLL patient with diffuse mediastinal lymphadenopathy who was diagnosed with metastatic bladder cancer without a primary bladder tumor mass on imaging.

CASE DESCRIPTION

A 60-year-old man with a 60 pack-year smoking history, alcoholic cirrhosis, and a 5-year history of stage 1 CLL presented with 3 months of progressive shortness of breath; persistent cough; chills; hemoptysis; and a steady weight loss of 35 lbs. Notably, he had no bladder symptoms. Initial labs showed leukocytosis of 35.8k with a lymphocytic predominance. Screening low-dose chest CT was positive for diffuse mediastinal lymphadenopathy. Subsequent PET/CT revealed numerous hypermetabolic lymph nodes in the neck, mediastinum, left hilum, and right periaortic abdominal region. CT Chest, Abdomen, Pelvis revealed progressive lymphadenopathy as seen in prior imaging, stable pulmonary nodules up to 4 mm in size, and splenomegaly. No distant primary sites, including of the bladder, were identified. Mediastinal lymph node biopsy confirmed metastatic poorly differentiated carcinoma with immunohistochemical staining negative for p40, p63, CK20, TTF-1, Napsin A, CDX2, CA19- 9, Calretinin, and D2-40 and positive for CK7, GATA3, Ber-EP4, and Uroplakin, supporting bladder as primary origin. Urology deferred a cystoscopy given his lack of urinary symptoms and positive biopsy and was started on Carboplatin/Gemcitabine for his metastatic disease. He was ineligible for Cisplatin given his cirrhosis and hearing impairment.

DISCUSSION

In patients with CLL, new onset mediastinal lymphadenopathy is concerning for disease progression and possible transformation to a diffuse b-cell lymphoma. However, this symptom has a broad differential, including primary lung carcinomas, sarcomas, and metastatic disease. While our patient’s PET/CT and pan-CT failed to identify a distant primary site, maintaining a low clinical suspicion for metastatic disease and doing a thorough work-up was paramount. Only through immunohistochemical staining were we able to diagnosis this patient with urothelial carcinoma.

CONCLUSIONS

Biopsy with immunohistochemical staining and maintaining a low suspicion for worsening lymphadenopathy can identify unusually presenting urothelial carcinomas in CLL patients.

INTRODUCTION

Lymphadenopathy in Chronic Lymphocytic Leukemia (CLL) is a very common feature. However, sudden increase in lymphadenopathy or other symptoms like weight loss should be evaluated for possible metastatic malignancy. We describe a CLL patient with diffuse mediastinal lymphadenopathy who was diagnosed with metastatic bladder cancer without a primary bladder tumor mass on imaging.

CASE DESCRIPTION

A 60-year-old man with a 60 pack-year smoking history, alcoholic cirrhosis, and a 5-year history of stage 1 CLL presented with 3 months of progressive shortness of breath; persistent cough; chills; hemoptysis; and a steady weight loss of 35 lbs. Notably, he had no bladder symptoms. Initial labs showed leukocytosis of 35.8k with a lymphocytic predominance. Screening low-dose chest CT was positive for diffuse mediastinal lymphadenopathy. Subsequent PET/CT revealed numerous hypermetabolic lymph nodes in the neck, mediastinum, left hilum, and right periaortic abdominal region. CT Chest, Abdomen, Pelvis revealed progressive lymphadenopathy as seen in prior imaging, stable pulmonary nodules up to 4 mm in size, and splenomegaly. No distant primary sites, including of the bladder, were identified. Mediastinal lymph node biopsy confirmed metastatic poorly differentiated carcinoma with immunohistochemical staining negative for p40, p63, CK20, TTF-1, Napsin A, CDX2, CA19- 9, Calretinin, and D2-40 and positive for CK7, GATA3, Ber-EP4, and Uroplakin, supporting bladder as primary origin. Urology deferred a cystoscopy given his lack of urinary symptoms and positive biopsy and was started on Carboplatin/Gemcitabine for his metastatic disease. He was ineligible for Cisplatin given his cirrhosis and hearing impairment.

DISCUSSION

In patients with CLL, new onset mediastinal lymphadenopathy is concerning for disease progression and possible transformation to a diffuse b-cell lymphoma. However, this symptom has a broad differential, including primary lung carcinomas, sarcomas, and metastatic disease. While our patient’s PET/CT and pan-CT failed to identify a distant primary site, maintaining a low clinical suspicion for metastatic disease and doing a thorough work-up was paramount. Only through immunohistochemical staining were we able to diagnosis this patient with urothelial carcinoma.

CONCLUSIONS

Biopsy with immunohistochemical staining and maintaining a low suspicion for worsening lymphadenopathy can identify unusually presenting urothelial carcinomas in CLL patients.

INTRODUCTION

Follicular thyroid cancer (FTC) is a common endocrine malignancy that is mainly treated with surgical resection. Few prior studies have investigated the optimal type of surgery for this FTC, particularly at a national registry level. The aim of this study is to examine the differences between surgical subtypes in the management of FTC.

METHODS

Patients from the Surveillance, Epidemiology, and End Results (SEER) database who were diagnosed with FTC between 2000-2020 were selected. The surgeries were categorized into sublobectomy, lobectomy, subtotal thyroidectomy, or thyroidectomy groups based on the surgical procedure performed. Additional variables were collected including age, sex, race, stage, radiation status, time to treatment, household income, and population size. Kaplan-Meier, Chi-square and logistic regression analyses were performed.

RESULTS

A total of 9,983 patients were included. Using Kaplan-Meier, there was improved survival for patients that received surgery (p<0.001). Patients who underwent lobectomy had greater survival than all groups (p<0.001) while thyroidectomy had greater survival compared to sub-lobectomy (p=0.015). On Chi-square, differences at one- and five-year survival were present between surgical groups (p=0.022 and p<0.001, respectively). However, logistic regression showed no survival difference between surgery type at one- and five-years. Additional findings include regional and distal staging having worse survival at one- and five-years (p’s<0.001) while median household income >$75,000 and receipt of radiation improved survival at one-year (p’s<0.05). Household income >$75,000 and radiation status no longer improved survival at five-years. Patients living outside metropolitan areas showed an improved survival at fiveyears (p=0.036).

CONCLUSIONS

The results of the preliminary Kaplan- Meier and Chi-square analysis showed that there are significant differences in survival between different surgery subtypes. However, after controlling for multiple variables, no survival differences were observed between surgical types. Despite minimal differences in FTC survival based on the type of surgical intervention, clinical factors like stage and radiation and socioeconomic factors like household income and population size may influence FTC survival. Identifying and controlling for these variables should be considered in future research on FTC.

INTRODUCTION

Follicular thyroid cancer (FTC) is a common endocrine malignancy that is mainly treated with surgical resection. Few prior studies have investigated the optimal type of surgery for this FTC, particularly at a national registry level. The aim of this study is to examine the differences between surgical subtypes in the management of FTC.

METHODS

Patients from the Surveillance, Epidemiology, and End Results (SEER) database who were diagnosed with FTC between 2000-2020 were selected. The surgeries were categorized into sublobectomy, lobectomy, subtotal thyroidectomy, or thyroidectomy groups based on the surgical procedure performed. Additional variables were collected including age, sex, race, stage, radiation status, time to treatment, household income, and population size. Kaplan-Meier, Chi-square and logistic regression analyses were performed.

RESULTS

A total of 9,983 patients were included. Using Kaplan-Meier, there was improved survival for patients that received surgery (p<0.001). Patients who underwent lobectomy had greater survival than all groups (p<0.001) while thyroidectomy had greater survival compared to sub-lobectomy (p=0.015). On Chi-square, differences at one- and five-year survival were present between surgical groups (p=0.022 and p<0.001, respectively). However, logistic regression showed no survival difference between surgery type at one- and five-years. Additional findings include regional and distal staging having worse survival at one- and five-years (p’s<0.001) while median household income >$75,000 and receipt of radiation improved survival at one-year (p’s<0.05). Household income >$75,000 and radiation status no longer improved survival at five-years. Patients living outside metropolitan areas showed an improved survival at fiveyears (p=0.036).

CONCLUSIONS

The results of the preliminary Kaplan- Meier and Chi-square analysis showed that there are significant differences in survival between different surgery subtypes. However, after controlling for multiple variables, no survival differences were observed between surgical types. Despite minimal differences in FTC survival based on the type of surgical intervention, clinical factors like stage and radiation and socioeconomic factors like household income and population size may influence FTC survival. Identifying and controlling for these variables should be considered in future research on FTC.

INTRODUCTION

Follicular thyroid cancer (FTC) is a common endocrine malignancy that is mainly treated with surgical resection. Few prior studies have investigated the optimal type of surgery for this FTC, particularly at a national registry level. The aim of this study is to examine the differences between surgical subtypes in the management of FTC.

METHODS

Patients from the Surveillance, Epidemiology, and End Results (SEER) database who were diagnosed with FTC between 2000-2020 were selected. The surgeries were categorized into sublobectomy, lobectomy, subtotal thyroidectomy, or thyroidectomy groups based on the surgical procedure performed. Additional variables were collected including age, sex, race, stage, radiation status, time to treatment, household income, and population size. Kaplan-Meier, Chi-square and logistic regression analyses were performed.

RESULTS

A total of 9,983 patients were included. Using Kaplan-Meier, there was improved survival for patients that received surgery (p<0.001). Patients who underwent lobectomy had greater survival than all groups (p<0.001) while thyroidectomy had greater survival compared to sub-lobectomy (p=0.015). On Chi-square, differences at one- and five-year survival were present between surgical groups (p=0.022 and p<0.001, respectively). However, logistic regression showed no survival difference between surgery type at one- and five-years. Additional findings include regional and distal staging having worse survival at one- and five-years (p’s<0.001) while median household income >$75,000 and receipt of radiation improved survival at one-year (p’s<0.05). Household income >$75,000 and radiation status no longer improved survival at five-years. Patients living outside metropolitan areas showed an improved survival at fiveyears (p=0.036).

CONCLUSIONS

The results of the preliminary Kaplan- Meier and Chi-square analysis showed that there are significant differences in survival between different surgery subtypes. However, after controlling for multiple variables, no survival differences were observed between surgical types. Despite minimal differences in FTC survival based on the type of surgical intervention, clinical factors like stage and radiation and socioeconomic factors like household income and population size may influence FTC survival. Identifying and controlling for these variables should be considered in future research on FTC.

BACKGROUND

Ewing sarcoma (EWS) is a malignancy which primarily arises in adolescence and has been studied extensively in this population. Much less is www.mdedge.com/fedprac/avaho SEPTEMBER 2023 • S23 known about the rare patient cohort over the age of 40 at diagnosis. In this study, we describe the survival outcomes and clinical characteristics of this population.

METHODS

This retrospective cohort study utilized the National Cancer Database (NCDB) to identify 4600 patients diagnosed between 2004 through 2019. Of these patients, 4058 were under the age of 40 and 542 were over 40. Multivariate Cox regression models and Kaplan- Meier curves were used to estimate survival from diagnosis to death between age groups. Chi-square tests were used to compare demographic and socioeconomic patient characteristics. IBM SPSS version 27.0 was used. p<0.05 was used to indicate statistical significance.

RESULTS

EWS patients older than 40 experienced worse survival outcomes compared to patients under the age of 40. 5-year survival was 43.5% for older patients vs. 64.5% for younger patients (p<0.05). A multivariate Cox proportional hazards model showed that age was independently associated with inferior survival. (HR 2.23; p<0.05). EWS patients over the age of 40 were more likely to have tumors originating from the vertebral column (16.2% vs. 9.6%; p<0.05), cranium (5.5% vs. 4.7%; p<0.05), and had a higher rate of axial tumors (43.3% vs. 32.4%; p<0.05) compared to patients under 40. Additionally, patients older than 40 experienced a significantly longer delay between the date of diagnosis and initiation of systemic treatment (29.85 days vs. 19.37 days; p<0.05). Despite presenting with larger tumors , older patients were less likely to undergo a surgical procedure of the primary site (47.6% vs. 52.2%; p<0.05) and had higher rates of micro- and macroscopic residual tumor following surgical resection.

CONCLUSIONS

An age over 40 is associated with decreased survival for patients with EWS. Due to the rarity of EWS in this cohort, the optimal role of systemic treatment remains unknown and has yet to be clearly elucidated. Consequently, our findings suggest that older patients receive disparities in treatment which may be contributing to decreased survival rates.

BACKGROUND

Ewing sarcoma (EWS) is a malignancy which primarily arises in adolescence and has been studied extensively in this population. Much less is www.mdedge.com/fedprac/avaho SEPTEMBER 2023 • S23 known about the rare patient cohort over the age of 40 at diagnosis. In this study, we describe the survival outcomes and clinical characteristics of this population.

METHODS

This retrospective cohort study utilized the National Cancer Database (NCDB) to identify 4600 patients diagnosed between 2004 through 2019. Of these patients, 4058 were under the age of 40 and 542 were over 40. Multivariate Cox regression models and Kaplan- Meier curves were used to estimate survival from diagnosis to death between age groups. Chi-square tests were used to compare demographic and socioeconomic patient characteristics. IBM SPSS version 27.0 was used. p<0.05 was used to indicate statistical significance.

RESULTS

EWS patients older than 40 experienced worse survival outcomes compared to patients under the age of 40. 5-year survival was 43.5% for older patients vs. 64.5% for younger patients (p<0.05). A multivariate Cox proportional hazards model showed that age was independently associated with inferior survival. (HR 2.23; p<0.05). EWS patients over the age of 40 were more likely to have tumors originating from the vertebral column (16.2% vs. 9.6%; p<0.05), cranium (5.5% vs. 4.7%; p<0.05), and had a higher rate of axial tumors (43.3% vs. 32.4%; p<0.05) compared to patients under 40. Additionally, patients older than 40 experienced a significantly longer delay between the date of diagnosis and initiation of systemic treatment (29.85 days vs. 19.37 days; p<0.05). Despite presenting with larger tumors , older patients were less likely to undergo a surgical procedure of the primary site (47.6% vs. 52.2%; p<0.05) and had higher rates of micro- and macroscopic residual tumor following surgical resection.

CONCLUSIONS

An age over 40 is associated with decreased survival for patients with EWS. Due to the rarity of EWS in this cohort, the optimal role of systemic treatment remains unknown and has yet to be clearly elucidated. Consequently, our findings suggest that older patients receive disparities in treatment which may be contributing to decreased survival rates.

BACKGROUND

Ewing sarcoma (EWS) is a malignancy which primarily arises in adolescence and has been studied extensively in this population. Much less is www.mdedge.com/fedprac/avaho SEPTEMBER 2023 • S23 known about the rare patient cohort over the age of 40 at diagnosis. In this study, we describe the survival outcomes and clinical characteristics of this population.

METHODS

This retrospective cohort study utilized the National Cancer Database (NCDB) to identify 4600 patients diagnosed between 2004 through 2019. Of these patients, 4058 were under the age of 40 and 542 were over 40. Multivariate Cox regression models and Kaplan- Meier curves were used to estimate survival from diagnosis to death between age groups. Chi-square tests were used to compare demographic and socioeconomic patient characteristics. IBM SPSS version 27.0 was used. p<0.05 was used to indicate statistical significance.

RESULTS

EWS patients older than 40 experienced worse survival outcomes compared to patients under the age of 40. 5-year survival was 43.5% for older patients vs. 64.5% for younger patients (p<0.05). A multivariate Cox proportional hazards model showed that age was independently associated with inferior survival. (HR 2.23; p<0.05). EWS patients over the age of 40 were more likely to have tumors originating from the vertebral column (16.2% vs. 9.6%; p<0.05), cranium (5.5% vs. 4.7%; p<0.05), and had a higher rate of axial tumors (43.3% vs. 32.4%; p<0.05) compared to patients under 40. Additionally, patients older than 40 experienced a significantly longer delay between the date of diagnosis and initiation of systemic treatment (29.85 days vs. 19.37 days; p<0.05). Despite presenting with larger tumors , older patients were less likely to undergo a surgical procedure of the primary site (47.6% vs. 52.2%; p<0.05) and had higher rates of micro- and macroscopic residual tumor following surgical resection.

CONCLUSIONS

An age over 40 is associated with decreased survival for patients with EWS. Due to the rarity of EWS in this cohort, the optimal role of systemic treatment remains unknown and has yet to be clearly elucidated. Consequently, our findings suggest that older patients receive disparities in treatment which may be contributing to decreased survival rates.

INTRODUCTION

Medulloblastoma (MB) is rarely seen in adulthood. Treatment guidelines are derived from studies of the pediatric population, results favoring the Packer regimen (cisplatin plus cyclophosphamide or lomustine plus vincristine). MB rarely has extraneural metastases, especially the bone marrow.

CASE PRESENTATION

A 32-year-old female with a past medical history of cerebellar MB confirmed on surgical pathology status post resection, weekly radiation and vincristine treatment presented to us in clinic to re-establish care. She was lost to follow-up 9 months after initial diagnosis and wished to continue treatment. She was started on Lomustine, Cisplatin and Vincristine after discussion with our colleagues at MSKCC, where she had received her initial treatment. After cycle three, she developed intractable bone pain and pancytopenia. Bone marrow biopsy revealed metastasis of Sonic Hedgehog Desmoplastic/nodular variant MB. PET and CT imaging confirmed metastatic disease in the bone marrow and repeat MRI brain showed abnormal nodular enhancement. CSF analysis to assess for spinal metastasis was negative. The patient was started on Temozolomide, Irinotecan and Bevacizumab with significant improvement in bone pain and radiological improvement noted on PET and CT scans. After cycle six, the patient had increased bone pain and repeat FDG-PET showed increased uptake, however, she continued to receive treatment and her pain has improved off narcotics.

DISCUSSION

We highlight a case of adult MB in the bone marrow responsive to temozolomide, irinotecan and bevacizumab. We conducted a literature search using PubMed, Medline and Web of Science between 1990 to 2022. In 2021, COG Phase 2 screening trial showed bevacizumab, temozolamide/irinotecan therapy significantly reduced the risk of death with recurrent MBs, two studies included patients up to 21 and 23 years of age. Other modalities showing some response include Vincristine plus cyclophosphamide as well as high dose carboplatin, thiotepa and etoposide alongside autologous SCT. Vismodegib has also shown varied response of 15 months in two adults with extraneural MB metastasis. Given the unique entity of adult MB and extraneural metastasis, limitations include small sample and lack of generalizability.

CONCLUSIONS

Extraneural metastasis of MB yields a poor prognosis. Future considerations include randomized trials to establish efficacy of Temozolomide, Irinotecan plus Bevacizumab in this population.

INTRODUCTION

Medulloblastoma (MB) is rarely seen in adulthood. Treatment guidelines are derived from studies of the pediatric population, results favoring the Packer regimen (cisplatin plus cyclophosphamide or lomustine plus vincristine). MB rarely has extraneural metastases, especially the bone marrow.

CASE PRESENTATION

A 32-year-old female with a past medical history of cerebellar MB confirmed on surgical pathology status post resection, weekly radiation and vincristine treatment presented to us in clinic to re-establish care. She was lost to follow-up 9 months after initial diagnosis and wished to continue treatment. She was started on Lomustine, Cisplatin and Vincristine after discussion with our colleagues at MSKCC, where she had received her initial treatment. After cycle three, she developed intractable bone pain and pancytopenia. Bone marrow biopsy revealed metastasis of Sonic Hedgehog Desmoplastic/nodular variant MB. PET and CT imaging confirmed metastatic disease in the bone marrow and repeat MRI brain showed abnormal nodular enhancement. CSF analysis to assess for spinal metastasis was negative. The patient was started on Temozolomide, Irinotecan and Bevacizumab with significant improvement in bone pain and radiological improvement noted on PET and CT scans. After cycle six, the patient had increased bone pain and repeat FDG-PET showed increased uptake, however, she continued to receive treatment and her pain has improved off narcotics.

DISCUSSION

We highlight a case of adult MB in the bone marrow responsive to temozolomide, irinotecan and bevacizumab. We conducted a literature search using PubMed, Medline and Web of Science between 1990 to 2022. In 2021, COG Phase 2 screening trial showed bevacizumab, temozolamide/irinotecan therapy significantly reduced the risk of death with recurrent MBs, two studies included patients up to 21 and 23 years of age. Other modalities showing some response include Vincristine plus cyclophosphamide as well as high dose carboplatin, thiotepa and etoposide alongside autologous SCT. Vismodegib has also shown varied response of 15 months in two adults with extraneural MB metastasis. Given the unique entity of adult MB and extraneural metastasis, limitations include small sample and lack of generalizability.

CONCLUSIONS

Extraneural metastasis of MB yields a poor prognosis. Future considerations include randomized trials to establish efficacy of Temozolomide, Irinotecan plus Bevacizumab in this population.

INTRODUCTION

Medulloblastoma (MB) is rarely seen in adulthood. Treatment guidelines are derived from studies of the pediatric population, results favoring the Packer regimen (cisplatin plus cyclophosphamide or lomustine plus vincristine). MB rarely has extraneural metastases, especially the bone marrow.

CASE PRESENTATION

A 32-year-old female with a past medical history of cerebellar MB confirmed on surgical pathology status post resection, weekly radiation and vincristine treatment presented to us in clinic to re-establish care. She was lost to follow-up 9 months after initial diagnosis and wished to continue treatment. She was started on Lomustine, Cisplatin and Vincristine after discussion with our colleagues at MSKCC, where she had received her initial treatment. After cycle three, she developed intractable bone pain and pancytopenia. Bone marrow biopsy revealed metastasis of Sonic Hedgehog Desmoplastic/nodular variant MB. PET and CT imaging confirmed metastatic disease in the bone marrow and repeat MRI brain showed abnormal nodular enhancement. CSF analysis to assess for spinal metastasis was negative. The patient was started on Temozolomide, Irinotecan and Bevacizumab with significant improvement in bone pain and radiological improvement noted on PET and CT scans. After cycle six, the patient had increased bone pain and repeat FDG-PET showed increased uptake, however, she continued to receive treatment and her pain has improved off narcotics.

DISCUSSION

We highlight a case of adult MB in the bone marrow responsive to temozolomide, irinotecan and bevacizumab. We conducted a literature search using PubMed, Medline and Web of Science between 1990 to 2022. In 2021, COG Phase 2 screening trial showed bevacizumab, temozolamide/irinotecan therapy significantly reduced the risk of death with recurrent MBs, two studies included patients up to 21 and 23 years of age. Other modalities showing some response include Vincristine plus cyclophosphamide as well as high dose carboplatin, thiotepa and etoposide alongside autologous SCT. Vismodegib has also shown varied response of 15 months in two adults with extraneural MB metastasis. Given the unique entity of adult MB and extraneural metastasis, limitations include small sample and lack of generalizability.

CONCLUSIONS

Extraneural metastasis of MB yields a poor prognosis. Future considerations include randomized trials to establish efficacy of Temozolomide, Irinotecan plus Bevacizumab in this population.

BACKGROUND/PURPOSE

Tumor lysis syndrome (TLS) occurs when malignant cells rapidly break down. This may lead to hyperuricemia, hyperkalemia, hyperphosphatemia, and/or hypocalcemia. Rasburicase reduces uric acid in cancer patients undergoing anti-cancer therapy. However, caution is required as rasburicase is contraindicated for patients with glucose- 6-phosphate dehydrogenase (G6PD) deficiency due to the increased risk of hemolysis. G6PD deficiency is more prevalent among African Americans (AA), affecting approximately 12% of this population. The FDA recommends testing for G6PD deficiency in higher risk groups before administering rasburicase.

METHODS

A retrospective analysis was conducted at the Louis Stokes Cleveland VAMC from February 1, 2018, to January 31, 2023 addressing appropriate use of rasburicase and incidence of G6PD deficiency and hemolysis. Appropriate use was defined by: TLS (2 or more: uric acid ≥ 8 or 25% increase; K+ ≥ 6.0 or 25% increase; Phos > 4.5mg/dL, or 25% increase; or calcium < 7, or 25% decrease, from baseline) or at high risk for TLS (CLL: venetoclax use w/lymph node > 10cm or WBC > 25k and elevated uric acid; AML: WBC > 100k; ALL: WBC > 100k and LDH 2x ULN; Burkitt lymphoma: LDH 2x ULN).

RESULTS

50 patients were identified who received rasburicase. 21/50 (42%) did not meet criteria for appropriate use. 44/50 (88%) underwent G6PD testing. The average time from G6PD testing order to obtaining the results was 3.4 days; 18/50 patients (36%) had G6PD resulted prior to rasburicase administration, and 26 patients (52%) received rasburicase prior to G6PD results. Overall, 13/50 (26%) were AA. Of the AA pts, 12/13 (92%) were tested for G6PD. Of these 12, 1/12 was found to be G6PD deficient and this patient experienced G6PD deficiency-induced hemolysis after rasburicase. None of the non-AA pts (0/31) tested were found to be G6PD deficient.

IMPLICATIONS

There was a high (42%) level of inappropriate use of rasburicase. G6PD deficiency was uncommon and only found in the AA population. To reduce inappropriate use, rasburicase orders will be restricted to medical oncology. G6PD testing will be limited to AA pts, with pathology to develop a rapid turnaround time for results prior to rasburicase administration to prevent hemolysis.

BACKGROUND/PURPOSE

Tumor lysis syndrome (TLS) occurs when malignant cells rapidly break down. This may lead to hyperuricemia, hyperkalemia, hyperphosphatemia, and/or hypocalcemia. Rasburicase reduces uric acid in cancer patients undergoing anti-cancer therapy. However, caution is required as rasburicase is contraindicated for patients with glucose- 6-phosphate dehydrogenase (G6PD) deficiency due to the increased risk of hemolysis. G6PD deficiency is more prevalent among African Americans (AA), affecting approximately 12% of this population. The FDA recommends testing for G6PD deficiency in higher risk groups before administering rasburicase.

METHODS

A retrospective analysis was conducted at the Louis Stokes Cleveland VAMC from February 1, 2018, to January 31, 2023 addressing appropriate use of rasburicase and incidence of G6PD deficiency and hemolysis. Appropriate use was defined by: TLS (2 or more: uric acid ≥ 8 or 25% increase; K+ ≥ 6.0 or 25% increase; Phos > 4.5mg/dL, or 25% increase; or calcium < 7, or 25% decrease, from baseline) or at high risk for TLS (CLL: venetoclax use w/lymph node > 10cm or WBC > 25k and elevated uric acid; AML: WBC > 100k; ALL: WBC > 100k and LDH 2x ULN; Burkitt lymphoma: LDH 2x ULN).

RESULTS

50 patients were identified who received rasburicase. 21/50 (42%) did not meet criteria for appropriate use. 44/50 (88%) underwent G6PD testing. The average time from G6PD testing order to obtaining the results was 3.4 days; 18/50 patients (36%) had G6PD resulted prior to rasburicase administration, and 26 patients (52%) received rasburicase prior to G6PD results. Overall, 13/50 (26%) were AA. Of the AA pts, 12/13 (92%) were tested for G6PD. Of these 12, 1/12 was found to be G6PD deficient and this patient experienced G6PD deficiency-induced hemolysis after rasburicase. None of the non-AA pts (0/31) tested were found to be G6PD deficient.

IMPLICATIONS

There was a high (42%) level of inappropriate use of rasburicase. G6PD deficiency was uncommon and only found in the AA population. To reduce inappropriate use, rasburicase orders will be restricted to medical oncology. G6PD testing will be limited to AA pts, with pathology to develop a rapid turnaround time for results prior to rasburicase administration to prevent hemolysis.

BACKGROUND/PURPOSE

Tumor lysis syndrome (TLS) occurs when malignant cells rapidly break down. This may lead to hyperuricemia, hyperkalemia, hyperphosphatemia, and/or hypocalcemia. Rasburicase reduces uric acid in cancer patients undergoing anti-cancer therapy. However, caution is required as rasburicase is contraindicated for patients with glucose- 6-phosphate dehydrogenase (G6PD) deficiency due to the increased risk of hemolysis. G6PD deficiency is more prevalent among African Americans (AA), affecting approximately 12% of this population. The FDA recommends testing for G6PD deficiency in higher risk groups before administering rasburicase.

METHODS

A retrospective analysis was conducted at the Louis Stokes Cleveland VAMC from February 1, 2018, to January 31, 2023 addressing appropriate use of rasburicase and incidence of G6PD deficiency and hemolysis. Appropriate use was defined by: TLS (2 or more: uric acid ≥ 8 or 25% increase; K+ ≥ 6.0 or 25% increase; Phos > 4.5mg/dL, or 25% increase; or calcium < 7, or 25% decrease, from baseline) or at high risk for TLS (CLL: venetoclax use w/lymph node > 10cm or WBC > 25k and elevated uric acid; AML: WBC > 100k; ALL: WBC > 100k and LDH 2x ULN; Burkitt lymphoma: LDH 2x ULN).

RESULTS

50 patients were identified who received rasburicase. 21/50 (42%) did not meet criteria for appropriate use. 44/50 (88%) underwent G6PD testing. The average time from G6PD testing order to obtaining the results was 3.4 days; 18/50 patients (36%) had G6PD resulted prior to rasburicase administration, and 26 patients (52%) received rasburicase prior to G6PD results. Overall, 13/50 (26%) were AA. Of the AA pts, 12/13 (92%) were tested for G6PD. Of these 12, 1/12 was found to be G6PD deficient and this patient experienced G6PD deficiency-induced hemolysis after rasburicase. None of the non-AA pts (0/31) tested were found to be G6PD deficient.

IMPLICATIONS

There was a high (42%) level of inappropriate use of rasburicase. G6PD deficiency was uncommon and only found in the AA population. To reduce inappropriate use, rasburicase orders will be restricted to medical oncology. G6PD testing will be limited to AA pts, with pathology to develop a rapid turnaround time for results prior to rasburicase administration to prevent hemolysis.