Blood type linked to higher risk for early onset stroke

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Individuals with type A blood have a 16% higher risk for early onset stroke (EOS) than those with other blood types, new research shows.

Conversely, results from a meta-analysis of nearly 17,000 cases of ischemic stroke in adults younger than 60 years showed that having type O blood reduced the risk for EOS by 12%.

In addition, the associations with risk were significantly stronger in EOS than in those with late-onset stroke (LOS), pointing to a stronger role for prothrombotic factors in younger patients, the researchers noted.

“What this is telling us is that maybe what makes you susceptible to stroke as a young adult is the blood type, which is really giving you a much higher risk of clotting and stroke compared to later onset,” coinvestigator Braxton Mitchell, PhD, professor of medicine and epidemiology and public health at the University of Maryland, Baltimore, said in an interview.

The findings were published online in Neurology.
 

Strong association

The genome-wide association study (GWAS) was done as part of the Genetics of Early Onset Ischemic Stroke Consortium, a collaboration of 48 different studies across North America, Europe, Japan, Pakistan, and Australia. It assessed early onset ischemic stroke in patients aged 18-59 years.

Researchers included data from 16,927 patients with stroke. Of these, 5,825 had a stroke before age 60, defined as early onset. GWAS results were also examined for nearly 600,000 individuals without stroke.

Results showed two genetic variants tied to blood types A and O emerged as highly associated with risk for early stroke.

Researchers found that the protective effects of type O were significantly stronger with EOS vs. LOS (odds ratio [OR], 0.88 vs. 0.96, respectively; P = .001). Likewise, the association between type A and increased EOS risk was significantly stronger than that found in LOS (OR, 1.16 vs. 1.05; P = .005).

Using polygenic risk scores, the investigators also found that the greater genetic risk for venous thromboembolism, another prothrombotic condition, was more strongly associated with EOS compared with LOS (P = .008).

Previous studies have shown a link between stroke risk and variants of the ABO gene, which determines blood type. The new analysis suggests that type A and O gene variants represent nearly all of those genetically linked with early stroke, the researchers noted.

While the findings point to blood type as a risk factor for stroke in younger people, Dr. Mitchell cautions that “at the moment, blood group does not have implications for preventive care.”

“The risk of stroke due to blood type is smaller than other risk factors that we know about, like smoking and hypertension,” he said. “I would be much more worried about these other risk factors, especially because those may be modifiable.”

He noted the next step in the study is to assess how blood type interacts with other known risk factors to raise stroke risk.

“There may be a subset of people where, if you have blood type A and you have some of these other risk factors, it’s possible that you may be at particularly high risk,” Dr. Mitchell said.
 

More research needed on younger patients

In an accompanying editorial, Jennifer Juhl Majersik, MD, associate professor of neurology at the University of Utah, Salt Lake City, and Paul Lacaze, PhD, associate professor and head of the public health genomics program at Monash University, Australia, noted that the study fills a gap in stroke research, which often focuses mostly on older individuals.

 

 

“In approximately 40% of people with EOS, the stroke is cryptogenic, and there is scant data from clinical trials to guide the selection of preventative strategies in this population, as people with EOS are often excluded from trials,” Dr. Majersik and Dr. Lacaze wrote.

“This work has deepened our understanding of EOS pathophysiology,” they added.

The editorialists noted that future research can build on the results from this analysis, “with the goal of a more precise understanding of stroke pathophysiology, leading to targeted preventative treatments for EOS and a reduction in disability in patients’ most productive years.”

Dr. Mitchell echoed the call for greater inclusion of young patients with stroke in clinical trials.

“As we’re learning, stroke in older folks isn’t the same as stroke in younger people,” he said. “There are many shared risk factors but there are also some that are different ... so there really is a need to include younger people.”

A version of this article first appeared on Medscape.com.

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Individuals with type A blood have a 16% higher risk for early onset stroke (EOS) than those with other blood types, new research shows.

Conversely, results from a meta-analysis of nearly 17,000 cases of ischemic stroke in adults younger than 60 years showed that having type O blood reduced the risk for EOS by 12%.

In addition, the associations with risk were significantly stronger in EOS than in those with late-onset stroke (LOS), pointing to a stronger role for prothrombotic factors in younger patients, the researchers noted.

“What this is telling us is that maybe what makes you susceptible to stroke as a young adult is the blood type, which is really giving you a much higher risk of clotting and stroke compared to later onset,” coinvestigator Braxton Mitchell, PhD, professor of medicine and epidemiology and public health at the University of Maryland, Baltimore, said in an interview.

The findings were published online in Neurology.
 

Strong association

The genome-wide association study (GWAS) was done as part of the Genetics of Early Onset Ischemic Stroke Consortium, a collaboration of 48 different studies across North America, Europe, Japan, Pakistan, and Australia. It assessed early onset ischemic stroke in patients aged 18-59 years.

Researchers included data from 16,927 patients with stroke. Of these, 5,825 had a stroke before age 60, defined as early onset. GWAS results were also examined for nearly 600,000 individuals without stroke.

Results showed two genetic variants tied to blood types A and O emerged as highly associated with risk for early stroke.

Researchers found that the protective effects of type O were significantly stronger with EOS vs. LOS (odds ratio [OR], 0.88 vs. 0.96, respectively; P = .001). Likewise, the association between type A and increased EOS risk was significantly stronger than that found in LOS (OR, 1.16 vs. 1.05; P = .005).

Using polygenic risk scores, the investigators also found that the greater genetic risk for venous thromboembolism, another prothrombotic condition, was more strongly associated with EOS compared with LOS (P = .008).

Previous studies have shown a link between stroke risk and variants of the ABO gene, which determines blood type. The new analysis suggests that type A and O gene variants represent nearly all of those genetically linked with early stroke, the researchers noted.

While the findings point to blood type as a risk factor for stroke in younger people, Dr. Mitchell cautions that “at the moment, blood group does not have implications for preventive care.”

“The risk of stroke due to blood type is smaller than other risk factors that we know about, like smoking and hypertension,” he said. “I would be much more worried about these other risk factors, especially because those may be modifiable.”

He noted the next step in the study is to assess how blood type interacts with other known risk factors to raise stroke risk.

“There may be a subset of people where, if you have blood type A and you have some of these other risk factors, it’s possible that you may be at particularly high risk,” Dr. Mitchell said.
 

More research needed on younger patients

In an accompanying editorial, Jennifer Juhl Majersik, MD, associate professor of neurology at the University of Utah, Salt Lake City, and Paul Lacaze, PhD, associate professor and head of the public health genomics program at Monash University, Australia, noted that the study fills a gap in stroke research, which often focuses mostly on older individuals.

 

 

“In approximately 40% of people with EOS, the stroke is cryptogenic, and there is scant data from clinical trials to guide the selection of preventative strategies in this population, as people with EOS are often excluded from trials,” Dr. Majersik and Dr. Lacaze wrote.

“This work has deepened our understanding of EOS pathophysiology,” they added.

The editorialists noted that future research can build on the results from this analysis, “with the goal of a more precise understanding of stroke pathophysiology, leading to targeted preventative treatments for EOS and a reduction in disability in patients’ most productive years.”

Dr. Mitchell echoed the call for greater inclusion of young patients with stroke in clinical trials.

“As we’re learning, stroke in older folks isn’t the same as stroke in younger people,” he said. “There are many shared risk factors but there are also some that are different ... so there really is a need to include younger people.”

A version of this article first appeared on Medscape.com.

Individuals with type A blood have a 16% higher risk for early onset stroke (EOS) than those with other blood types, new research shows.

Conversely, results from a meta-analysis of nearly 17,000 cases of ischemic stroke in adults younger than 60 years showed that having type O blood reduced the risk for EOS by 12%.

In addition, the associations with risk were significantly stronger in EOS than in those with late-onset stroke (LOS), pointing to a stronger role for prothrombotic factors in younger patients, the researchers noted.

“What this is telling us is that maybe what makes you susceptible to stroke as a young adult is the blood type, which is really giving you a much higher risk of clotting and stroke compared to later onset,” coinvestigator Braxton Mitchell, PhD, professor of medicine and epidemiology and public health at the University of Maryland, Baltimore, said in an interview.

The findings were published online in Neurology.
 

Strong association

The genome-wide association study (GWAS) was done as part of the Genetics of Early Onset Ischemic Stroke Consortium, a collaboration of 48 different studies across North America, Europe, Japan, Pakistan, and Australia. It assessed early onset ischemic stroke in patients aged 18-59 years.

Researchers included data from 16,927 patients with stroke. Of these, 5,825 had a stroke before age 60, defined as early onset. GWAS results were also examined for nearly 600,000 individuals without stroke.

Results showed two genetic variants tied to blood types A and O emerged as highly associated with risk for early stroke.

Researchers found that the protective effects of type O were significantly stronger with EOS vs. LOS (odds ratio [OR], 0.88 vs. 0.96, respectively; P = .001). Likewise, the association between type A and increased EOS risk was significantly stronger than that found in LOS (OR, 1.16 vs. 1.05; P = .005).

Using polygenic risk scores, the investigators also found that the greater genetic risk for venous thromboembolism, another prothrombotic condition, was more strongly associated with EOS compared with LOS (P = .008).

Previous studies have shown a link between stroke risk and variants of the ABO gene, which determines blood type. The new analysis suggests that type A and O gene variants represent nearly all of those genetically linked with early stroke, the researchers noted.

While the findings point to blood type as a risk factor for stroke in younger people, Dr. Mitchell cautions that “at the moment, blood group does not have implications for preventive care.”

“The risk of stroke due to blood type is smaller than other risk factors that we know about, like smoking and hypertension,” he said. “I would be much more worried about these other risk factors, especially because those may be modifiable.”

He noted the next step in the study is to assess how blood type interacts with other known risk factors to raise stroke risk.

“There may be a subset of people where, if you have blood type A and you have some of these other risk factors, it’s possible that you may be at particularly high risk,” Dr. Mitchell said.
 

More research needed on younger patients

In an accompanying editorial, Jennifer Juhl Majersik, MD, associate professor of neurology at the University of Utah, Salt Lake City, and Paul Lacaze, PhD, associate professor and head of the public health genomics program at Monash University, Australia, noted that the study fills a gap in stroke research, which often focuses mostly on older individuals.

 

 

“In approximately 40% of people with EOS, the stroke is cryptogenic, and there is scant data from clinical trials to guide the selection of preventative strategies in this population, as people with EOS are often excluded from trials,” Dr. Majersik and Dr. Lacaze wrote.

“This work has deepened our understanding of EOS pathophysiology,” they added.

The editorialists noted that future research can build on the results from this analysis, “with the goal of a more precise understanding of stroke pathophysiology, leading to targeted preventative treatments for EOS and a reduction in disability in patients’ most productive years.”

Dr. Mitchell echoed the call for greater inclusion of young patients with stroke in clinical trials.

“As we’re learning, stroke in older folks isn’t the same as stroke in younger people,” he said. “There are many shared risk factors but there are also some that are different ... so there really is a need to include younger people.”

A version of this article first appeared on Medscape.com.

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Academic/Research Facility Utilization and Survival Outcomes in Osteosarcoma: An NCDB Analysis

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Background

Previous studies have reported that treatment at academic/research facilities is associated with improved survival in cancer patients. The objective of this study was to investigate the impact of treatment facility type on overall survival for patients presenting with osteosarcoma.

Methods

The National Cancer Database (NCDB) was used to identify patients diagnosed with Osteosarcoma from 2004 to 2018. Facility types were identified as assigned by the Commission on Cancer Accreditation program. Data was analyzed using SPSS and statistical significance was set at P = .05.

Results

Of 2085 patients queried, 39.6% were treated at an academic/research program. The stage-adjusted difference in median survival between academic/research and non-academic programs was found to be statistically significant on log-rank comparison (P < .001). At each NCDB analytic stage (stage I-IV), academic/research programs were associated with decreased hazard and improved median survival. A Cox proportional hazards model showed a decreased likelihood of mortality in patients with osteosarcoma who underwent treatment at an academic/research program (HR, 0.882; 95% CI, .802-.969; P = .009). Chi-square testing revealed that patients at academic/research programs were more likely than those at non-academic/research centers to have private insurance, less likely to have Medicare, and more likely to live in counties of > 1 million people. These facilities were also more likely to have undergone Medicaid expansion in 2014. (P < .05). Patients at non-academic/research programs were more likely to have advanced disease (stage III and IV) and higher comorbidity scores. Additionally, they were less likely to receive surgery and/or chemotherapy at the institution in which they were diagnosed. (P < .05).

Conclusions

This study showed that Osteosarcoma patients treated in an academic/research program facility experienced increased survival compared with non-academic/research facilities. Patients at academic/research facilities tend to have less comorbidities, have private insurance, and present with more treatable disease. Despite these favorable prognostic factors, the data suggest an intrinsic benefit to being treated at an academic/research facility.

 

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Background

Previous studies have reported that treatment at academic/research facilities is associated with improved survival in cancer patients. The objective of this study was to investigate the impact of treatment facility type on overall survival for patients presenting with osteosarcoma.

Methods

The National Cancer Database (NCDB) was used to identify patients diagnosed with Osteosarcoma from 2004 to 2018. Facility types were identified as assigned by the Commission on Cancer Accreditation program. Data was analyzed using SPSS and statistical significance was set at P = .05.

Results

Of 2085 patients queried, 39.6% were treated at an academic/research program. The stage-adjusted difference in median survival between academic/research and non-academic programs was found to be statistically significant on log-rank comparison (P < .001). At each NCDB analytic stage (stage I-IV), academic/research programs were associated with decreased hazard and improved median survival. A Cox proportional hazards model showed a decreased likelihood of mortality in patients with osteosarcoma who underwent treatment at an academic/research program (HR, 0.882; 95% CI, .802-.969; P = .009). Chi-square testing revealed that patients at academic/research programs were more likely than those at non-academic/research centers to have private insurance, less likely to have Medicare, and more likely to live in counties of > 1 million people. These facilities were also more likely to have undergone Medicaid expansion in 2014. (P < .05). Patients at non-academic/research programs were more likely to have advanced disease (stage III and IV) and higher comorbidity scores. Additionally, they were less likely to receive surgery and/or chemotherapy at the institution in which they were diagnosed. (P < .05).

Conclusions

This study showed that Osteosarcoma patients treated in an academic/research program facility experienced increased survival compared with non-academic/research facilities. Patients at academic/research facilities tend to have less comorbidities, have private insurance, and present with more treatable disease. Despite these favorable prognostic factors, the data suggest an intrinsic benefit to being treated at an academic/research facility.

 

Background

Previous studies have reported that treatment at academic/research facilities is associated with improved survival in cancer patients. The objective of this study was to investigate the impact of treatment facility type on overall survival for patients presenting with osteosarcoma.

Methods

The National Cancer Database (NCDB) was used to identify patients diagnosed with Osteosarcoma from 2004 to 2018. Facility types were identified as assigned by the Commission on Cancer Accreditation program. Data was analyzed using SPSS and statistical significance was set at P = .05.

Results

Of 2085 patients queried, 39.6% were treated at an academic/research program. The stage-adjusted difference in median survival between academic/research and non-academic programs was found to be statistically significant on log-rank comparison (P < .001). At each NCDB analytic stage (stage I-IV), academic/research programs were associated with decreased hazard and improved median survival. A Cox proportional hazards model showed a decreased likelihood of mortality in patients with osteosarcoma who underwent treatment at an academic/research program (HR, 0.882; 95% CI, .802-.969; P = .009). Chi-square testing revealed that patients at academic/research programs were more likely than those at non-academic/research centers to have private insurance, less likely to have Medicare, and more likely to live in counties of > 1 million people. These facilities were also more likely to have undergone Medicaid expansion in 2014. (P < .05). Patients at non-academic/research programs were more likely to have advanced disease (stage III and IV) and higher comorbidity scores. Additionally, they were less likely to receive surgery and/or chemotherapy at the institution in which they were diagnosed. (P < .05).

Conclusions

This study showed that Osteosarcoma patients treated in an academic/research program facility experienced increased survival compared with non-academic/research facilities. Patients at academic/research facilities tend to have less comorbidities, have private insurance, and present with more treatable disease. Despite these favorable prognostic factors, the data suggest an intrinsic benefit to being treated at an academic/research facility.

 

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Financial Toxicity in Colorectal Cancer Patient Who Received Localized Treatment in the Veterans Affairs Health System

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Purpose

To describe patient-reported financial toxicity for patients who received localized colorectal cancer (CRC) treatment in the Veterans Health Administration (VHA).

Background

CRC is the 2nd leading cause of cancer-related death. In the private sector, many patients suffer economic hardship from CRC and its treatment. This leads to financial toxicity, or the negative impact of medical expenses, which is a strong independent predictor of quality of life. In the VHA patients access cancer care based on a sliding fee scale; however, there is a knowledge gap regarding financial toxicity for CRC patients in the VHA whose out of pocket costs have largely been subsidized.

Methods

We performed a descriptive, retrospective analysis of a survey administered at a VHA facility to patients with colorectal cancer who received localized treatment (ie, surgery or chemoradiotherapy). The survey consisted of 49 items assessing several clinical and psychosocial domains including subjective financial burden and use of financial coping strategies. Additionally, we used the validated Confusion, Hubbub and Order Scale (CHAOS) measure, which was designed to assess the level of confusion and disorganization in homes.

Results

Between November 2015 and September 2016, we mailed surveys to 265 patients diagnosed with CRC, 133 responded, for a response rate of 50%. For financial strain, 24% (n=32) of participants reported reduced spending on basics like food or clothing to pay for their cancer treatment, 17% (n=23) reported using all or a portion of their savings to pay for their cancer care,14% (n=18) noted borrowing money or using a credit card to pay for care, and 9% (n=12) of participants noted they did not fill a prescription because it was too expensive.

Conclusions/Implications

Despite policies to reduce out-of-pocket costs for VHA patients with CRC, patients reported significant financial toxicity. In the continued movement for value-based care centered on whole person care delivery, identifying persistent financial toxicity for vulnerable cancer patients is important data as we try and improve the infrastructure to impact quality of life and healthcare delivery for this population.

 

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Purpose

To describe patient-reported financial toxicity for patients who received localized colorectal cancer (CRC) treatment in the Veterans Health Administration (VHA).

Background

CRC is the 2nd leading cause of cancer-related death. In the private sector, many patients suffer economic hardship from CRC and its treatment. This leads to financial toxicity, or the negative impact of medical expenses, which is a strong independent predictor of quality of life. In the VHA patients access cancer care based on a sliding fee scale; however, there is a knowledge gap regarding financial toxicity for CRC patients in the VHA whose out of pocket costs have largely been subsidized.

Methods

We performed a descriptive, retrospective analysis of a survey administered at a VHA facility to patients with colorectal cancer who received localized treatment (ie, surgery or chemoradiotherapy). The survey consisted of 49 items assessing several clinical and psychosocial domains including subjective financial burden and use of financial coping strategies. Additionally, we used the validated Confusion, Hubbub and Order Scale (CHAOS) measure, which was designed to assess the level of confusion and disorganization in homes.

Results

Between November 2015 and September 2016, we mailed surveys to 265 patients diagnosed with CRC, 133 responded, for a response rate of 50%. For financial strain, 24% (n=32) of participants reported reduced spending on basics like food or clothing to pay for their cancer treatment, 17% (n=23) reported using all or a portion of their savings to pay for their cancer care,14% (n=18) noted borrowing money or using a credit card to pay for care, and 9% (n=12) of participants noted they did not fill a prescription because it was too expensive.

Conclusions/Implications

Despite policies to reduce out-of-pocket costs for VHA patients with CRC, patients reported significant financial toxicity. In the continued movement for value-based care centered on whole person care delivery, identifying persistent financial toxicity for vulnerable cancer patients is important data as we try and improve the infrastructure to impact quality of life and healthcare delivery for this population.

 

Purpose

To describe patient-reported financial toxicity for patients who received localized colorectal cancer (CRC) treatment in the Veterans Health Administration (VHA).

Background

CRC is the 2nd leading cause of cancer-related death. In the private sector, many patients suffer economic hardship from CRC and its treatment. This leads to financial toxicity, or the negative impact of medical expenses, which is a strong independent predictor of quality of life. In the VHA patients access cancer care based on a sliding fee scale; however, there is a knowledge gap regarding financial toxicity for CRC patients in the VHA whose out of pocket costs have largely been subsidized.

Methods

We performed a descriptive, retrospective analysis of a survey administered at a VHA facility to patients with colorectal cancer who received localized treatment (ie, surgery or chemoradiotherapy). The survey consisted of 49 items assessing several clinical and psychosocial domains including subjective financial burden and use of financial coping strategies. Additionally, we used the validated Confusion, Hubbub and Order Scale (CHAOS) measure, which was designed to assess the level of confusion and disorganization in homes.

Results

Between November 2015 and September 2016, we mailed surveys to 265 patients diagnosed with CRC, 133 responded, for a response rate of 50%. For financial strain, 24% (n=32) of participants reported reduced spending on basics like food or clothing to pay for their cancer treatment, 17% (n=23) reported using all or a portion of their savings to pay for their cancer care,14% (n=18) noted borrowing money or using a credit card to pay for care, and 9% (n=12) of participants noted they did not fill a prescription because it was too expensive.

Conclusions/Implications

Despite policies to reduce out-of-pocket costs for VHA patients with CRC, patients reported significant financial toxicity. In the continued movement for value-based care centered on whole person care delivery, identifying persistent financial toxicity for vulnerable cancer patients is important data as we try and improve the infrastructure to impact quality of life and healthcare delivery for this population.

 

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Impact of Insurance Status on Survival in Hurthle Cell Carcinoma: A National Cancer Database (NCDB) Analysis

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Background

Hurthle cell carcinoma (HCC), also known as oxyphilic adenocarcinoma, is a rare malignancy characterized by the presence of mitochondrion-rich, eosinophilic epithelial cells known as Hurthle cells. HCC is a variant of follicular thyroid cancer and can metastasize more aggressively than other thyroid malignancies. The purpose of this study is to identify how insurance status impacts median survival time in patients with HCC.

Methods

Using the NCDB, we identified 10,378 patients diagnosed with HCC between 2004 and 2016 using ICD-O-3 histology code 8290. The cohort was analyzed to identify differences in survival outcomes based on the insurance status of the patient during treatment. The 4 categories of insurance identified were uninsured, private insurance, Medicaid, and Medicare. Univariate analysis was performed assessing patient length of survival for each insurance subtype. Data were analyzed using SPSS and statistical significance was set at P = .05.

Results 

We identified statistically significant differences (P < .001) in survival outcomes between privately insured patients and patients with Medicaid or Medicare. Privately insured patients had the highest rates of median survival with 150.9 months, while patients on Medicare had the lowest rates of median survival with 108.1 months. Medicaid and uninsured patients had a median survival rates of 134.5 months and 141.9 months, respectively. 40.8% of privately insured patients presented at stage I, while 20.8% of Medicare patients presented at stage I. Patients with private insurance had the lowest rate of presenting with stage IV disease at 5.0%, which was dramatically different from patients with Medicare that presented with stage IV HCC at a rate of 13.0%.

Conclusions

This study shows the discrepancies of survival in patients with HCC based on insurance coverage. HCC patients with private insurance have significantly longer survival outcomes than patients on Medicaid and Medicare. We hypothesize that privately insured patients are more likely to seek treatment earlier and receive a higher level of care. Privately insured patients were also less likely to present with Stage IV HCC than patients with other insurance statuses. Future directions should analyze how treatment type affects survival outcomes.

 

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Background

Hurthle cell carcinoma (HCC), also known as oxyphilic adenocarcinoma, is a rare malignancy characterized by the presence of mitochondrion-rich, eosinophilic epithelial cells known as Hurthle cells. HCC is a variant of follicular thyroid cancer and can metastasize more aggressively than other thyroid malignancies. The purpose of this study is to identify how insurance status impacts median survival time in patients with HCC.

Methods

Using the NCDB, we identified 10,378 patients diagnosed with HCC between 2004 and 2016 using ICD-O-3 histology code 8290. The cohort was analyzed to identify differences in survival outcomes based on the insurance status of the patient during treatment. The 4 categories of insurance identified were uninsured, private insurance, Medicaid, and Medicare. Univariate analysis was performed assessing patient length of survival for each insurance subtype. Data were analyzed using SPSS and statistical significance was set at P = .05.

Results 

We identified statistically significant differences (P < .001) in survival outcomes between privately insured patients and patients with Medicaid or Medicare. Privately insured patients had the highest rates of median survival with 150.9 months, while patients on Medicare had the lowest rates of median survival with 108.1 months. Medicaid and uninsured patients had a median survival rates of 134.5 months and 141.9 months, respectively. 40.8% of privately insured patients presented at stage I, while 20.8% of Medicare patients presented at stage I. Patients with private insurance had the lowest rate of presenting with stage IV disease at 5.0%, which was dramatically different from patients with Medicare that presented with stage IV HCC at a rate of 13.0%.

Conclusions

This study shows the discrepancies of survival in patients with HCC based on insurance coverage. HCC patients with private insurance have significantly longer survival outcomes than patients on Medicaid and Medicare. We hypothesize that privately insured patients are more likely to seek treatment earlier and receive a higher level of care. Privately insured patients were also less likely to present with Stage IV HCC than patients with other insurance statuses. Future directions should analyze how treatment type affects survival outcomes.

 

Background

Hurthle cell carcinoma (HCC), also known as oxyphilic adenocarcinoma, is a rare malignancy characterized by the presence of mitochondrion-rich, eosinophilic epithelial cells known as Hurthle cells. HCC is a variant of follicular thyroid cancer and can metastasize more aggressively than other thyroid malignancies. The purpose of this study is to identify how insurance status impacts median survival time in patients with HCC.

Methods

Using the NCDB, we identified 10,378 patients diagnosed with HCC between 2004 and 2016 using ICD-O-3 histology code 8290. The cohort was analyzed to identify differences in survival outcomes based on the insurance status of the patient during treatment. The 4 categories of insurance identified were uninsured, private insurance, Medicaid, and Medicare. Univariate analysis was performed assessing patient length of survival for each insurance subtype. Data were analyzed using SPSS and statistical significance was set at P = .05.

Results 

We identified statistically significant differences (P < .001) in survival outcomes between privately insured patients and patients with Medicaid or Medicare. Privately insured patients had the highest rates of median survival with 150.9 months, while patients on Medicare had the lowest rates of median survival with 108.1 months. Medicaid and uninsured patients had a median survival rates of 134.5 months and 141.9 months, respectively. 40.8% of privately insured patients presented at stage I, while 20.8% of Medicare patients presented at stage I. Patients with private insurance had the lowest rate of presenting with stage IV disease at 5.0%, which was dramatically different from patients with Medicare that presented with stage IV HCC at a rate of 13.0%.

Conclusions

This study shows the discrepancies of survival in patients with HCC based on insurance coverage. HCC patients with private insurance have significantly longer survival outcomes than patients on Medicaid and Medicare. We hypothesize that privately insured patients are more likely to seek treatment earlier and receive a higher level of care. Privately insured patients were also less likely to present with Stage IV HCC than patients with other insurance statuses. Future directions should analyze how treatment type affects survival outcomes.

 

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Shortened radiotherapy for endometrial cancer looks safe, questions remain

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Postoperative radiotherapy is a mainstay in the treatment of endometrial cancer, but the typical 5-week regimen can be time consuming and expensive. A pilot study found that delivery of approximately the same dose over just two and a half weeks, known as hypofractionation, had good short-term toxicity outcomes.

It isn’t yet clear if the protocol will be as effective as the standard course, and long-term side effects may still be an issue.

Nevertheless, shortening the duration of radiotherapy could have benefits, especially in advanced uterine cancer, where chemotherapy is employed against distant metastases. Following surgery, there is a risk of both local recurrence and distant metastasis, complicating the choice of initial treatment. “Chemo can be several months long and radiation is typically several weeks. Therefore a shortened radiation schedule may have potential benefits, especially if there is an opportunity for this to be delivered earlier without delaying or interrupting chemotherapy, for example,” said lead study author Eric Leung, MD, associate professor of radiation oncology at the University of Toronto’s Sunnybrook Health Sciences Centre.

The research was published in JAMA Oncology.

Delivery of hypofractionation is tricky, according to Dr. Leung. “Gynecological cancer patients were treated with hypofractionation radiation to the pelvis which included the vagina, paravaginal tissues and pelvic lymph nodes. With this relatively large pelvic volume with surrounding normal tissues, this requires a highly focused radiation treatment with advanced technology,” said Dr. Leung. The study protocol employed stereotactic technique to deliver 30 Gy in 5 fractions.

Hypofractionation could be beneficial in reduction of travel time and time spent in hospital, as well as reducing financial burden and increasing quality of life. These benefits have taken on a larger role in the context of the COVID-19 pandemic.

Although the findings are encouraging, they are preliminary, according to Vonetta Williams, MD, PhD, who wrote an accompanying editorial. “I would caution that all they’ve done is presented preliminary toxicity data, so we don’t have any proof yet that it is equally effective (compared to standard protocol), and their study cannot answer that at any rate because it was not designed to answer that question,” Dr. Williams said in an interview. She noted that long-term follow-up is needed to measure bowel dysfunction, sexual dysfunction, vaginal stenosis, and other side effects.

It is also uncertain whether hypofractionated doses are actually equivalent to the standard dose. “We know that they’re roughly equivalent, but that is very much a question if they are equivalent in terms of efficacy. I don’t know that I would be confident that they are. That’s probably what would give most radiation oncologists pause, because we don’t have any data to say that it is (equivalent). Although it would be nice to shorten treatment, and I think it would certainly be better for patients, I want to caution that we want to do so once we know what the toxicity and the outcomes really are,” said Dr. Williams.

The researchers enrolled 61 patients with a median age of 66 years. Thirty-nine had endometrioid adenocarcinoma, 15 had serous or clear cell, 3 had carcinosarcoma, and 4 had dedifferentiated disease. Sixteen patients underwent sequential chemotherapy, and 9 underwent additional vault brachytherapy. Over a median follow-up of 9 months, 54% had a worst gastrointestinal side effect of grade 1, while 13% had a worst side effect of grade 2. Among worst genitourinary side effects, 41% had grade 1 and 3% had grade 2. One patient had acute grade 3 diarrhea at fraction 5, but this resolved at follow-up. One patient had diarrhea scores that were both clinically and statistically significantly worse than baseline at fraction 5, and this improved at follow-up.

Patient-reported quality of life outcomes were generally good. Of all measures, only diarrhea was clinically and statistically worse by fraction 5, and improvement was seen at 6 weeks and 3 months. Global health status was consistent throughout treatment and follow-up. There was no change in sexual and vaginal symptoms.

The study authors reported grants, consulting, and personal fees from a variety of pharmaceutical companies. Dr. Williams reported having no disclosures.

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Postoperative radiotherapy is a mainstay in the treatment of endometrial cancer, but the typical 5-week regimen can be time consuming and expensive. A pilot study found that delivery of approximately the same dose over just two and a half weeks, known as hypofractionation, had good short-term toxicity outcomes.

It isn’t yet clear if the protocol will be as effective as the standard course, and long-term side effects may still be an issue.

Nevertheless, shortening the duration of radiotherapy could have benefits, especially in advanced uterine cancer, where chemotherapy is employed against distant metastases. Following surgery, there is a risk of both local recurrence and distant metastasis, complicating the choice of initial treatment. “Chemo can be several months long and radiation is typically several weeks. Therefore a shortened radiation schedule may have potential benefits, especially if there is an opportunity for this to be delivered earlier without delaying or interrupting chemotherapy, for example,” said lead study author Eric Leung, MD, associate professor of radiation oncology at the University of Toronto’s Sunnybrook Health Sciences Centre.

The research was published in JAMA Oncology.

Delivery of hypofractionation is tricky, according to Dr. Leung. “Gynecological cancer patients were treated with hypofractionation radiation to the pelvis which included the vagina, paravaginal tissues and pelvic lymph nodes. With this relatively large pelvic volume with surrounding normal tissues, this requires a highly focused radiation treatment with advanced technology,” said Dr. Leung. The study protocol employed stereotactic technique to deliver 30 Gy in 5 fractions.

Hypofractionation could be beneficial in reduction of travel time and time spent in hospital, as well as reducing financial burden and increasing quality of life. These benefits have taken on a larger role in the context of the COVID-19 pandemic.

Although the findings are encouraging, they are preliminary, according to Vonetta Williams, MD, PhD, who wrote an accompanying editorial. “I would caution that all they’ve done is presented preliminary toxicity data, so we don’t have any proof yet that it is equally effective (compared to standard protocol), and their study cannot answer that at any rate because it was not designed to answer that question,” Dr. Williams said in an interview. She noted that long-term follow-up is needed to measure bowel dysfunction, sexual dysfunction, vaginal stenosis, and other side effects.

It is also uncertain whether hypofractionated doses are actually equivalent to the standard dose. “We know that they’re roughly equivalent, but that is very much a question if they are equivalent in terms of efficacy. I don’t know that I would be confident that they are. That’s probably what would give most radiation oncologists pause, because we don’t have any data to say that it is (equivalent). Although it would be nice to shorten treatment, and I think it would certainly be better for patients, I want to caution that we want to do so once we know what the toxicity and the outcomes really are,” said Dr. Williams.

The researchers enrolled 61 patients with a median age of 66 years. Thirty-nine had endometrioid adenocarcinoma, 15 had serous or clear cell, 3 had carcinosarcoma, and 4 had dedifferentiated disease. Sixteen patients underwent sequential chemotherapy, and 9 underwent additional vault brachytherapy. Over a median follow-up of 9 months, 54% had a worst gastrointestinal side effect of grade 1, while 13% had a worst side effect of grade 2. Among worst genitourinary side effects, 41% had grade 1 and 3% had grade 2. One patient had acute grade 3 diarrhea at fraction 5, but this resolved at follow-up. One patient had diarrhea scores that were both clinically and statistically significantly worse than baseline at fraction 5, and this improved at follow-up.

Patient-reported quality of life outcomes were generally good. Of all measures, only diarrhea was clinically and statistically worse by fraction 5, and improvement was seen at 6 weeks and 3 months. Global health status was consistent throughout treatment and follow-up. There was no change in sexual and vaginal symptoms.

The study authors reported grants, consulting, and personal fees from a variety of pharmaceutical companies. Dr. Williams reported having no disclosures.

Postoperative radiotherapy is a mainstay in the treatment of endometrial cancer, but the typical 5-week regimen can be time consuming and expensive. A pilot study found that delivery of approximately the same dose over just two and a half weeks, known as hypofractionation, had good short-term toxicity outcomes.

It isn’t yet clear if the protocol will be as effective as the standard course, and long-term side effects may still be an issue.

Nevertheless, shortening the duration of radiotherapy could have benefits, especially in advanced uterine cancer, where chemotherapy is employed against distant metastases. Following surgery, there is a risk of both local recurrence and distant metastasis, complicating the choice of initial treatment. “Chemo can be several months long and radiation is typically several weeks. Therefore a shortened radiation schedule may have potential benefits, especially if there is an opportunity for this to be delivered earlier without delaying or interrupting chemotherapy, for example,” said lead study author Eric Leung, MD, associate professor of radiation oncology at the University of Toronto’s Sunnybrook Health Sciences Centre.

The research was published in JAMA Oncology.

Delivery of hypofractionation is tricky, according to Dr. Leung. “Gynecological cancer patients were treated with hypofractionation radiation to the pelvis which included the vagina, paravaginal tissues and pelvic lymph nodes. With this relatively large pelvic volume with surrounding normal tissues, this requires a highly focused radiation treatment with advanced technology,” said Dr. Leung. The study protocol employed stereotactic technique to deliver 30 Gy in 5 fractions.

Hypofractionation could be beneficial in reduction of travel time and time spent in hospital, as well as reducing financial burden and increasing quality of life. These benefits have taken on a larger role in the context of the COVID-19 pandemic.

Although the findings are encouraging, they are preliminary, according to Vonetta Williams, MD, PhD, who wrote an accompanying editorial. “I would caution that all they’ve done is presented preliminary toxicity data, so we don’t have any proof yet that it is equally effective (compared to standard protocol), and their study cannot answer that at any rate because it was not designed to answer that question,” Dr. Williams said in an interview. She noted that long-term follow-up is needed to measure bowel dysfunction, sexual dysfunction, vaginal stenosis, and other side effects.

It is also uncertain whether hypofractionated doses are actually equivalent to the standard dose. “We know that they’re roughly equivalent, but that is very much a question if they are equivalent in terms of efficacy. I don’t know that I would be confident that they are. That’s probably what would give most radiation oncologists pause, because we don’t have any data to say that it is (equivalent). Although it would be nice to shorten treatment, and I think it would certainly be better for patients, I want to caution that we want to do so once we know what the toxicity and the outcomes really are,” said Dr. Williams.

The researchers enrolled 61 patients with a median age of 66 years. Thirty-nine had endometrioid adenocarcinoma, 15 had serous or clear cell, 3 had carcinosarcoma, and 4 had dedifferentiated disease. Sixteen patients underwent sequential chemotherapy, and 9 underwent additional vault brachytherapy. Over a median follow-up of 9 months, 54% had a worst gastrointestinal side effect of grade 1, while 13% had a worst side effect of grade 2. Among worst genitourinary side effects, 41% had grade 1 and 3% had grade 2. One patient had acute grade 3 diarrhea at fraction 5, but this resolved at follow-up. One patient had diarrhea scores that were both clinically and statistically significantly worse than baseline at fraction 5, and this improved at follow-up.

Patient-reported quality of life outcomes were generally good. Of all measures, only diarrhea was clinically and statistically worse by fraction 5, and improvement was seen at 6 weeks and 3 months. Global health status was consistent throughout treatment and follow-up. There was no change in sexual and vaginal symptoms.

The study authors reported grants, consulting, and personal fees from a variety of pharmaceutical companies. Dr. Williams reported having no disclosures.

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Demographics in Early vs Late-Stage Laryngeal Squamous Cell Carcinoma: A NCDB Review

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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.

 

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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.

 

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Not Just Castleman Disease: an Elusive Diagnosis of TAFRO Syndrome

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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.

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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.

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Waldenstrom Macroglobulinemia Presenting With Schnitzler Syndrome

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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.

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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.

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Disparities in Palliative Care Utilization in Malignant Mixed Mullerian Tumor: A National Cancer Database (NCDB) Study

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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.

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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.

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Complete Remission of Metastatic Renal Cell Carcinoma after COVID-19 Vaccination

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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.

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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.

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