Federal Practitioner

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.

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.

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.

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.

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.

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.

Purpose 

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

Background 

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

Method/Analysis

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

Results 

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

Conclusions/Implications

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

Purpose 

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

Background 

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

Method/Analysis

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

Results 

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

Conclusions/Implications

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

Purpose 

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

Background 

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

Method/Analysis

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

Results 

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

Conclusions/Implications

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

Introduction

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

Case Report 

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

Discussion

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

Introduction

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

Case Report 

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

Discussion

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

Introduction

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

Case Report 

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

Discussion

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

Background

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

Case Report 

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

Results 

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

Background

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

Case Report 

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

Results 

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

Background

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

Case Report 

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

Results 

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

Study Purpose/Background

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

Methods/Design

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

Results 

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

Conclusions/Implications

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

Study Purpose/Background

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

Methods/Design

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

Results 

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

Conclusions/Implications

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

Study Purpose/Background

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

Methods/Design

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

Results 

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

Conclusions/Implications

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