Silberstein PT

Background: The prognostic implication of tumor location in breast cancer remains unclear. Previous studies suggested that inner and lower quadrant-located tumors were associated with decreased survival.

Methods: The NCDB was quired to identify AJCC clinical stage I-III first female breast cancer patients with unilateral disease who underwent breast-conserving surgery from 2010 to 2016. Three mutually exclusive groups were created based on tumor location, which included outer (upper and lower outer quadrants), central, and inner (upper and lower inner quadrants) zones of the breast. Clinical and demographic variables were obtained. Unadjusted survival differences were examined with Kaplan- Meier method. Multivariate Cox regression model was employed to examine the association between zone group and survival.

Results: 125,800 patients were identified including 83,558 (66.4%), 6,764 (5.4%), and 35,449 (28.2%) patients within the outer, central, and inner zones. There was evidence of a difference in age and tumor size based on site (P<0.001). It was also found that there was an association between tumor zone and each of the following (P<0.05): race and ethnicity, Charlson-Deyo score, insurance, income, education, facility type, laterality, histology, utilization of chemotherapy, ER status, PR status, and HER2 status. There was no relationship between site and unadjusted survival (P=0.905). After adjusting for all else, a 5.7% decreased the risk of death was found upon the comparison of the outer vs. inner zone of the breast (95% CI: 0.4%-11.8%; P=0.037). In general, older African American Medicaid patients with increased Charlson-Deyo scores and hormone receptor-negative breast cancers from lower-income areas had decreased survival.

Conclusion: Outer zone breast cancer has a more favorable survival advantage when compared with inner zone cancers. A finding that warrants re-evaluation of the management approach to inner zone breast cancer.

Background: The prognostic implication of tumor location in breast cancer remains unclear. Previous studies suggested that inner and lower quadrant-located tumors were associated with decreased survival.

Methods: The NCDB was quired to identify AJCC clinical stage I-III first female breast cancer patients with unilateral disease who underwent breast-conserving surgery from 2010 to 2016. Three mutually exclusive groups were created based on tumor location, which included outer (upper and lower outer quadrants), central, and inner (upper and lower inner quadrants) zones of the breast. Clinical and demographic variables were obtained. Unadjusted survival differences were examined with Kaplan- Meier method. Multivariate Cox regression model was employed to examine the association between zone group and survival.

Results: 125,800 patients were identified including 83,558 (66.4%), 6,764 (5.4%), and 35,449 (28.2%) patients within the outer, central, and inner zones. There was evidence of a difference in age and tumor size based on site (P<0.001). It was also found that there was an association between tumor zone and each of the following (P<0.05): race and ethnicity, Charlson-Deyo score, insurance, income, education, facility type, laterality, histology, utilization of chemotherapy, ER status, PR status, and HER2 status. There was no relationship between site and unadjusted survival (P=0.905). After adjusting for all else, a 5.7% decreased the risk of death was found upon the comparison of the outer vs. inner zone of the breast (95% CI: 0.4%-11.8%; P=0.037). In general, older African American Medicaid patients with increased Charlson-Deyo scores and hormone receptor-negative breast cancers from lower-income areas had decreased survival.

Conclusion: Outer zone breast cancer has a more favorable survival advantage when compared with inner zone cancers. A finding that warrants re-evaluation of the management approach to inner zone breast cancer.

Background: The prognostic implication of tumor location in breast cancer remains unclear. Previous studies suggested that inner and lower quadrant-located tumors were associated with decreased survival.

Methods: The NCDB was quired to identify AJCC clinical stage I-III first female breast cancer patients with unilateral disease who underwent breast-conserving surgery from 2010 to 2016. Three mutually exclusive groups were created based on tumor location, which included outer (upper and lower outer quadrants), central, and inner (upper and lower inner quadrants) zones of the breast. Clinical and demographic variables were obtained. Unadjusted survival differences were examined with Kaplan- Meier method. Multivariate Cox regression model was employed to examine the association between zone group and survival.

Results: 125,800 patients were identified including 83,558 (66.4%), 6,764 (5.4%), and 35,449 (28.2%) patients within the outer, central, and inner zones. There was evidence of a difference in age and tumor size based on site (P<0.001). It was also found that there was an association between tumor zone and each of the following (P<0.05): race and ethnicity, Charlson-Deyo score, insurance, income, education, facility type, laterality, histology, utilization of chemotherapy, ER status, PR status, and HER2 status. There was no relationship between site and unadjusted survival (P=0.905). After adjusting for all else, a 5.7% decreased the risk of death was found upon the comparison of the outer vs. inner zone of the breast (95% CI: 0.4%-11.8%; P=0.037). In general, older African American Medicaid patients with increased Charlson-Deyo scores and hormone receptor-negative breast cancers from lower-income areas had decreased survival.

Conclusion: Outer zone breast cancer has a more favorable survival advantage when compared with inner zone cancers. A finding that warrants re-evaluation of the management approach to inner zone breast cancer.

Background: Chronic Myelomonocytic leukemia (CMML) is a clonal hematopoietic malignancy and remains the most common overlapping myelodysplastic/ myeloproliferative neoplasm. Complications of CMML to acute monocytic leukemia transformation may include leukostasis, tumor lysis syndrome, and disseminated intravascular coagulation. We report a case that illustrates the disease characteristics and complications of CMML.

Case Report: A 76-year-old man, who was diagnosed 3 months prior to presentation with CMML, presented to the emergency department with dyspnea, altered mental status and right upper quadrant abdominal pain. His white count on admission was 314.5 × 109/L, which included 50 × 109/L blasts. His liver enzymes were elevated, and initial lactic acid was 12.5. No source of infection was found on imaging with Computed Tomography. Flow cytometry of peripheral blood revealed Acute Monocytic Leukemia. The diagnosis was consistent with CMML transformation to acute monocytic leukemia with hyperleukocytosis and leukostasis. He was started on cytoreduction therapy with hydroxyurea, leukapheresis, and decitabine chemotherapy. His symptoms improved with normalization of his white cell counts and he was discharged to concurrent hospice.

Discussion: Blast transformation (BT) to acute monocytic leukemia occurs in about 14% of patients diagnosed with CMML. 10-20% of newly diagnosed acute myeloid leukemia patients develop hyperleukocytosis (white cell counts greater than 100 x 109/L), a laboratory abnormality which may manifest clinically as leukostasis. Leukostasis, diagnosed empirically in a patient with BT who presents with respiratory and neurological symptoms, has 1-week mortality of 20%-40% if left untreated. Treatment of leukostasis includes leukapheresis, hydroxyurea, and induction chemotherapy. Recent studies have shown that leukapheresis reduces four-week mortality but does not affect long term mortality rate. More research is needed in understanding the pathophysiology of leukostasis, thus paving the way for novel therapeutic agents.

Background: Chronic Myelomonocytic leukemia (CMML) is a clonal hematopoietic malignancy and remains the most common overlapping myelodysplastic/ myeloproliferative neoplasm. Complications of CMML to acute monocytic leukemia transformation may include leukostasis, tumor lysis syndrome, and disseminated intravascular coagulation. We report a case that illustrates the disease characteristics and complications of CMML.

Case Report: A 76-year-old man, who was diagnosed 3 months prior to presentation with CMML, presented to the emergency department with dyspnea, altered mental status and right upper quadrant abdominal pain. His white count on admission was 314.5 × 109/L, which included 50 × 109/L blasts. His liver enzymes were elevated, and initial lactic acid was 12.5. No source of infection was found on imaging with Computed Tomography. Flow cytometry of peripheral blood revealed Acute Monocytic Leukemia. The diagnosis was consistent with CMML transformation to acute monocytic leukemia with hyperleukocytosis and leukostasis. He was started on cytoreduction therapy with hydroxyurea, leukapheresis, and decitabine chemotherapy. His symptoms improved with normalization of his white cell counts and he was discharged to concurrent hospice.

Discussion: Blast transformation (BT) to acute monocytic leukemia occurs in about 14% of patients diagnosed with CMML. 10-20% of newly diagnosed acute myeloid leukemia patients develop hyperleukocytosis (white cell counts greater than 100 x 109/L), a laboratory abnormality which may manifest clinically as leukostasis. Leukostasis, diagnosed empirically in a patient with BT who presents with respiratory and neurological symptoms, has 1-week mortality of 20%-40% if left untreated. Treatment of leukostasis includes leukapheresis, hydroxyurea, and induction chemotherapy. Recent studies have shown that leukapheresis reduces four-week mortality but does not affect long term mortality rate. More research is needed in understanding the pathophysiology of leukostasis, thus paving the way for novel therapeutic agents.

Background: Chronic Myelomonocytic leukemia (CMML) is a clonal hematopoietic malignancy and remains the most common overlapping myelodysplastic/ myeloproliferative neoplasm. Complications of CMML to acute monocytic leukemia transformation may include leukostasis, tumor lysis syndrome, and disseminated intravascular coagulation. We report a case that illustrates the disease characteristics and complications of CMML.

Case Report: A 76-year-old man, who was diagnosed 3 months prior to presentation with CMML, presented to the emergency department with dyspnea, altered mental status and right upper quadrant abdominal pain. His white count on admission was 314.5 × 109/L, which included 50 × 109/L blasts. His liver enzymes were elevated, and initial lactic acid was 12.5. No source of infection was found on imaging with Computed Tomography. Flow cytometry of peripheral blood revealed Acute Monocytic Leukemia. The diagnosis was consistent with CMML transformation to acute monocytic leukemia with hyperleukocytosis and leukostasis. He was started on cytoreduction therapy with hydroxyurea, leukapheresis, and decitabine chemotherapy. His symptoms improved with normalization of his white cell counts and he was discharged to concurrent hospice.

Discussion: Blast transformation (BT) to acute monocytic leukemia occurs in about 14% of patients diagnosed with CMML. 10-20% of newly diagnosed acute myeloid leukemia patients develop hyperleukocytosis (white cell counts greater than 100 x 109/L), a laboratory abnormality which may manifest clinically as leukostasis. Leukostasis, diagnosed empirically in a patient with BT who presents with respiratory and neurological symptoms, has 1-week mortality of 20%-40% if left untreated. Treatment of leukostasis includes leukapheresis, hydroxyurea, and induction chemotherapy. Recent studies have shown that leukapheresis reduces four-week mortality but does not affect long term mortality rate. More research is needed in understanding the pathophysiology of leukostasis, thus paving the way for novel therapeutic agents.

Background: Angioimmunoblastic T-cell lymphoma (AITL) is a rare and aggressive malignancy, representing approximately 18.5% of Peripheral T-cell lymphomas. The clinical presentation varies greatly, and literature describing this disease is limited. In this study we utilize the National Cancer Database (NCDB) to describe the patient characteristics, demographics, and overall survival of AITL.

Methods: The NCDB for non-Hodgkin’s lymphoma was used to identify 3,708 patients diagnosed with AITL between 2004 and 2016. We examined demographic information including age, race, gender, stage, and treatment modality. Kaplan-Meier analysis was used to analyze overall survival and compare survival by patient age, stage, and year of diagnosis. Bivariate and multivariate analysis was used to obtain hazard ratios and assess the association of patient characteristics and treatment methods with survival.

Results: The majority of AITL patients were white 87.8%, males 53%, with an average age of 67 years. 90% of patients received treatment, with 77.4% receiving chemotherapy, 10.8% receiving hematopoietic transplant, and 2.6% receiving radiation. B symptoms were present in 57% of patients. 43% were diagnosed with stage III disease and 43.5% at stage IV.

Median survival was 22 months (CI 19.7-24.4), with a 5- and 10-year survival of 30% and 22%. Median survival for patients aged 35-50 was 74 months, 50-65 was 37.7 months, 65-80 was 20.5 months, and for patients aged > 80 years old median survival was 6.4 months. Patients with stage I disease survived 35.1 months whereas those with stage IV disease survived 17.2 months. On multivariate analysis black race, increasing age, charleson-deyo comorbidity score, and stage at diagnoses were signi cantly associated with increased mortality risk. All forms of treatment, including chemotherapy, hematopoietic transplant, and radiation were all associated with improved survival.

Discussion: Our study found that the majority of AITL patients present with late stage disease, often with B symptoms, and have poor prognosis. We found a five-year survival of 30% for this malignancy when all stages of disease were combined. Knowledge of the patient characteristics, treatment modalities, and overall survival in AITL can serve to enhance the care of providers who encounter this uncommon diagnosis.

Background: Angioimmunoblastic T-cell lymphoma (AITL) is a rare and aggressive malignancy, representing approximately 18.5% of Peripheral T-cell lymphomas. The clinical presentation varies greatly, and literature describing this disease is limited. In this study we utilize the National Cancer Database (NCDB) to describe the patient characteristics, demographics, and overall survival of AITL.

Methods: The NCDB for non-Hodgkin’s lymphoma was used to identify 3,708 patients diagnosed with AITL between 2004 and 2016. We examined demographic information including age, race, gender, stage, and treatment modality. Kaplan-Meier analysis was used to analyze overall survival and compare survival by patient age, stage, and year of diagnosis. Bivariate and multivariate analysis was used to obtain hazard ratios and assess the association of patient characteristics and treatment methods with survival.

Results: The majority of AITL patients were white 87.8%, males 53%, with an average age of 67 years. 90% of patients received treatment, with 77.4% receiving chemotherapy, 10.8% receiving hematopoietic transplant, and 2.6% receiving radiation. B symptoms were present in 57% of patients. 43% were diagnosed with stage III disease and 43.5% at stage IV.

Median survival was 22 months (CI 19.7-24.4), with a 5- and 10-year survival of 30% and 22%. Median survival for patients aged 35-50 was 74 months, 50-65 was 37.7 months, 65-80 was 20.5 months, and for patients aged > 80 years old median survival was 6.4 months. Patients with stage I disease survived 35.1 months whereas those with stage IV disease survived 17.2 months. On multivariate analysis black race, increasing age, charleson-deyo comorbidity score, and stage at diagnoses were signi cantly associated with increased mortality risk. All forms of treatment, including chemotherapy, hematopoietic transplant, and radiation were all associated with improved survival.

Discussion: Our study found that the majority of AITL patients present with late stage disease, often with B symptoms, and have poor prognosis. We found a five-year survival of 30% for this malignancy when all stages of disease were combined. Knowledge of the patient characteristics, treatment modalities, and overall survival in AITL can serve to enhance the care of providers who encounter this uncommon diagnosis.

Background: Angioimmunoblastic T-cell lymphoma (AITL) is a rare and aggressive malignancy, representing approximately 18.5% of Peripheral T-cell lymphomas. The clinical presentation varies greatly, and literature describing this disease is limited. In this study we utilize the National Cancer Database (NCDB) to describe the patient characteristics, demographics, and overall survival of AITL.

Methods: The NCDB for non-Hodgkin’s lymphoma was used to identify 3,708 patients diagnosed with AITL between 2004 and 2016. We examined demographic information including age, race, gender, stage, and treatment modality. Kaplan-Meier analysis was used to analyze overall survival and compare survival by patient age, stage, and year of diagnosis. Bivariate and multivariate analysis was used to obtain hazard ratios and assess the association of patient characteristics and treatment methods with survival.

Results: The majority of AITL patients were white 87.8%, males 53%, with an average age of 67 years. 90% of patients received treatment, with 77.4% receiving chemotherapy, 10.8% receiving hematopoietic transplant, and 2.6% receiving radiation. B symptoms were present in 57% of patients. 43% were diagnosed with stage III disease and 43.5% at stage IV.

Median survival was 22 months (CI 19.7-24.4), with a 5- and 10-year survival of 30% and 22%. Median survival for patients aged 35-50 was 74 months, 50-65 was 37.7 months, 65-80 was 20.5 months, and for patients aged > 80 years old median survival was 6.4 months. Patients with stage I disease survived 35.1 months whereas those with stage IV disease survived 17.2 months. On multivariate analysis black race, increasing age, charleson-deyo comorbidity score, and stage at diagnoses were signi cantly associated with increased mortality risk. All forms of treatment, including chemotherapy, hematopoietic transplant, and radiation were all associated with improved survival.

Discussion: Our study found that the majority of AITL patients present with late stage disease, often with B symptoms, and have poor prognosis. We found a five-year survival of 30% for this malignancy when all stages of disease were combined. Knowledge of the patient characteristics, treatment modalities, and overall survival in AITL can serve to enhance the care of providers who encounter this uncommon diagnosis.

Background: Oligodendrogliomas represent about 12% of all brain tumors. Our goal was to compare the demographic factors of patients diagnosed with oligodendroglioma from 2004-2014 identified in the National Cancer Database (NCDB). We also examined the survival of patients based off of the number of their comorbidities.

Methods: We identified 7525 patients diagnosed with oligodendroglioma in the NCDB diagnosed between 2004-2014. Many demographic factors were examined such as age, gender, race, facility treated at, comorbidities, and surgery type. Between-insurance survival differences were estimated by the Kaplan-Meier method and associated log-rank tests; Tukey-Kramer adjusted P < .05 indicated statistical significance.

Results: More men were diagnosed with the tumor than females (55% vs 45%). Average age of patients at diagnosis was 43.5 years old. 66% of patients had private insurance, while 7% of patients were uninsured. 88.9% of patients were white, while 5.5% of patients were black. Patients that were treated at an academic/ research program were 32% of the sample size. 17% of the sample size were treated at a comprehensive community cancer program. Those with no comorbidities had the highest mean survival time of 111 months, those with one comorbidity had a mean survival time of 97 months, and those with two comorbidities had the lowest mean survival time of 75 months. 12.8% of patients had radical, total gross resection of tumor, lesion, or mass in their brain and 10% of patients had less than half of the lobe involved with the tumor resection. 20.1% of patients had systemic therapy after surgery. 59% of patients had no systemic therapy or surgery.

Conclusion: Our study shows men were affected more than women and that the mean age at diagnosis was 44 years old. The greater number of comorbidities a patient had, the lower the mean survival time was. Majority of patients were treated at an academic/research program. This is one of the largest studies to examine the demographics of patients with oligodendroglioma. Understanding who and how patients are affected can allow us to provide better resources and treatment.

Background: Oligodendrogliomas represent about 12% of all brain tumors. Our goal was to compare the demographic factors of patients diagnosed with oligodendroglioma from 2004-2014 identified in the National Cancer Database (NCDB). We also examined the survival of patients based off of the number of their comorbidities.

Methods: We identified 7525 patients diagnosed with oligodendroglioma in the NCDB diagnosed between 2004-2014. Many demographic factors were examined such as age, gender, race, facility treated at, comorbidities, and surgery type. Between-insurance survival differences were estimated by the Kaplan-Meier method and associated log-rank tests; Tukey-Kramer adjusted P < .05 indicated statistical significance.

Results: More men were diagnosed with the tumor than females (55% vs 45%). Average age of patients at diagnosis was 43.5 years old. 66% of patients had private insurance, while 7% of patients were uninsured. 88.9% of patients were white, while 5.5% of patients were black. Patients that were treated at an academic/ research program were 32% of the sample size. 17% of the sample size were treated at a comprehensive community cancer program. Those with no comorbidities had the highest mean survival time of 111 months, those with one comorbidity had a mean survival time of 97 months, and those with two comorbidities had the lowest mean survival time of 75 months. 12.8% of patients had radical, total gross resection of tumor, lesion, or mass in their brain and 10% of patients had less than half of the lobe involved with the tumor resection. 20.1% of patients had systemic therapy after surgery. 59% of patients had no systemic therapy or surgery.

Conclusion: Our study shows men were affected more than women and that the mean age at diagnosis was 44 years old. The greater number of comorbidities a patient had, the lower the mean survival time was. Majority of patients were treated at an academic/research program. This is one of the largest studies to examine the demographics of patients with oligodendroglioma. Understanding who and how patients are affected can allow us to provide better resources and treatment.

Background: Oligodendrogliomas represent about 12% of all brain tumors. Our goal was to compare the demographic factors of patients diagnosed with oligodendroglioma from 2004-2014 identified in the National Cancer Database (NCDB). We also examined the survival of patients based off of the number of their comorbidities.

Methods: We identified 7525 patients diagnosed with oligodendroglioma in the NCDB diagnosed between 2004-2014. Many demographic factors were examined such as age, gender, race, facility treated at, comorbidities, and surgery type. Between-insurance survival differences were estimated by the Kaplan-Meier method and associated log-rank tests; Tukey-Kramer adjusted P < .05 indicated statistical significance.

Results: More men were diagnosed with the tumor than females (55% vs 45%). Average age of patients at diagnosis was 43.5 years old. 66% of patients had private insurance, while 7% of patients were uninsured. 88.9% of patients were white, while 5.5% of patients were black. Patients that were treated at an academic/ research program were 32% of the sample size. 17% of the sample size were treated at a comprehensive community cancer program. Those with no comorbidities had the highest mean survival time of 111 months, those with one comorbidity had a mean survival time of 97 months, and those with two comorbidities had the lowest mean survival time of 75 months. 12.8% of patients had radical, total gross resection of tumor, lesion, or mass in their brain and 10% of patients had less than half of the lobe involved with the tumor resection. 20.1% of patients had systemic therapy after surgery. 59% of patients had no systemic therapy or surgery.

Conclusion: Our study shows men were affected more than women and that the mean age at diagnosis was 44 years old. The greater number of comorbidities a patient had, the lower the mean survival time was. Majority of patients were treated at an academic/research program. This is one of the largest studies to examine the demographics of patients with oligodendroglioma. Understanding who and how patients are affected can allow us to provide better resources and treatment.

Introduction: Primary Cardiac Sarcomas (PCS) are exceptionally rare malignancies, representing approximately 25% of all malignant primary cardiac tumors. Due to the rarity of these neoplasms, literature on the characteristics, optimal management, and survival outcomes in these patients is limited.

Methods: The National Cancer Database (NCDB) for soft tissue tumors was utilized to identify 826 adult patients diagnosed with tumors localized to the heart or pericardium from 2004 to 2016. Demographic information was obtained and Kaplan-Meier analysis was used to analyze overall survival of PCS. Bivariate analysis was performed with Cox proportional hazards regression models to obtain hazard ratios and assess the association of patient characteristics and treatment methods with survival.

Results: The majority of PCS patients were male (51.5%) and white (79.4%), with a mean age at diagnosis of 53 years. 41.2% were blood vessel tumors and 27.7% were sarcomas. Leiomyosarcoma and epithelial neoplasms each represented 5.2% of tumors, followed by synovial sarcomas (4.2%) and rhabdomyosarcomas (2.8%). The majority of patients were diagnosed with metastatic disease (43.2%) and received treatment (85.2%), most often with surgical resection (58.1%) or chemotherapy (57.2%).

Median overall survival was 10.9 months (95% CI: 9.6 – 12.1 months), with a cumulative survival at 1-year, 5-years, and 10-years of 27%, 10%, and 4%, respectively. Factors associated with signi cantly increased mortality (P<0.05), include increased age (HR 1.017), increased Charleson-Deyo comorbidity score (HR 1.284), and elevated stage and grade at diagnosis. Compared to blood vessel tumors, leiomyosarcoma (HR 0.696), fibroblastic (HR 0.579), osseous (HR 0.537), and fibrohystocytic (HR 0.485) histologies were associated with improved survival (P<0.05). Factors associated with signi cantly improved survival (P<0.05) included treatment by surgical resection (HR 0.500), radiation (HR 0.808), and chemotherapy (HR 0.738).

Conclusion: This is the largest study of PCS to date, and the first to analyze the NCDB. The majority of these neoplasms are blood vessel tumors and are often diagnosed at advanced stage and grade. Prognosis is poor, and all treatment modalities are associated with improved survival. Understanding of patient characteristics and overall survival is important in enhancing patient outcomes for this rare diagnosis.

Introduction: Primary Cardiac Sarcomas (PCS) are exceptionally rare malignancies, representing approximately 25% of all malignant primary cardiac tumors. Due to the rarity of these neoplasms, literature on the characteristics, optimal management, and survival outcomes in these patients is limited.

Methods: The National Cancer Database (NCDB) for soft tissue tumors was utilized to identify 826 adult patients diagnosed with tumors localized to the heart or pericardium from 2004 to 2016. Demographic information was obtained and Kaplan-Meier analysis was used to analyze overall survival of PCS. Bivariate analysis was performed with Cox proportional hazards regression models to obtain hazard ratios and assess the association of patient characteristics and treatment methods with survival.

Results: The majority of PCS patients were male (51.5%) and white (79.4%), with a mean age at diagnosis of 53 years. 41.2% were blood vessel tumors and 27.7% were sarcomas. Leiomyosarcoma and epithelial neoplasms each represented 5.2% of tumors, followed by synovial sarcomas (4.2%) and rhabdomyosarcomas (2.8%). The majority of patients were diagnosed with metastatic disease (43.2%) and received treatment (85.2%), most often with surgical resection (58.1%) or chemotherapy (57.2%).

Median overall survival was 10.9 months (95% CI: 9.6 – 12.1 months), with a cumulative survival at 1-year, 5-years, and 10-years of 27%, 10%, and 4%, respectively. Factors associated with signi cantly increased mortality (P<0.05), include increased age (HR 1.017), increased Charleson-Deyo comorbidity score (HR 1.284), and elevated stage and grade at diagnosis. Compared to blood vessel tumors, leiomyosarcoma (HR 0.696), fibroblastic (HR 0.579), osseous (HR 0.537), and fibrohystocytic (HR 0.485) histologies were associated with improved survival (P<0.05). Factors associated with signi cantly improved survival (P<0.05) included treatment by surgical resection (HR 0.500), radiation (HR 0.808), and chemotherapy (HR 0.738).

Conclusion: This is the largest study of PCS to date, and the first to analyze the NCDB. The majority of these neoplasms are blood vessel tumors and are often diagnosed at advanced stage and grade. Prognosis is poor, and all treatment modalities are associated with improved survival. Understanding of patient characteristics and overall survival is important in enhancing patient outcomes for this rare diagnosis.

Introduction: Primary Cardiac Sarcomas (PCS) are exceptionally rare malignancies, representing approximately 25% of all malignant primary cardiac tumors. Due to the rarity of these neoplasms, literature on the characteristics, optimal management, and survival outcomes in these patients is limited.

Methods: The National Cancer Database (NCDB) for soft tissue tumors was utilized to identify 826 adult patients diagnosed with tumors localized to the heart or pericardium from 2004 to 2016. Demographic information was obtained and Kaplan-Meier analysis was used to analyze overall survival of PCS. Bivariate analysis was performed with Cox proportional hazards regression models to obtain hazard ratios and assess the association of patient characteristics and treatment methods with survival.

Results: The majority of PCS patients were male (51.5%) and white (79.4%), with a mean age at diagnosis of 53 years. 41.2% were blood vessel tumors and 27.7% were sarcomas. Leiomyosarcoma and epithelial neoplasms each represented 5.2% of tumors, followed by synovial sarcomas (4.2%) and rhabdomyosarcomas (2.8%). The majority of patients were diagnosed with metastatic disease (43.2%) and received treatment (85.2%), most often with surgical resection (58.1%) or chemotherapy (57.2%).

Median overall survival was 10.9 months (95% CI: 9.6 – 12.1 months), with a cumulative survival at 1-year, 5-years, and 10-years of 27%, 10%, and 4%, respectively. Factors associated with signi cantly increased mortality (P<0.05), include increased age (HR 1.017), increased Charleson-Deyo comorbidity score (HR 1.284), and elevated stage and grade at diagnosis. Compared to blood vessel tumors, leiomyosarcoma (HR 0.696), fibroblastic (HR 0.579), osseous (HR 0.537), and fibrohystocytic (HR 0.485) histologies were associated with improved survival (P<0.05). Factors associated with signi cantly improved survival (P<0.05) included treatment by surgical resection (HR 0.500), radiation (HR 0.808), and chemotherapy (HR 0.738).

Conclusion: This is the largest study of PCS to date, and the first to analyze the NCDB. The majority of these neoplasms are blood vessel tumors and are often diagnosed at advanced stage and grade. Prognosis is poor, and all treatment modalities are associated with improved survival. Understanding of patient characteristics and overall survival is important in enhancing patient outcomes for this rare diagnosis.

Purpose: Lymph node yield is an independent prognostic factor for survival outcomes in patients receiving neck dissection for oral squamous cell carcinoma (SCC). This study investigates whether hospital case volume is associated with increased lymph node yield in neck dissection specimens.

Background: Guidelines recommending minimum number of lymph nodes that constitutes an adequate neck dissection have not been established. There is evidence that comprehensive neck dissections with improved lymph node yield ( 18 nodes) are associated with better survival outcomes. Recent studies have proposed this association to be a potential institutional-level quality metric. However, whetherinstitutional experience (reflected by case volume) influences the quality and comprehensiveness of neck dissection (reflected in node yield) remains unknown.

Methods: The National Cancer Database was used to identify 21,150 patients who underwent primary neck dissections for oral SCC diagnosed between 2004 and 2015. Average annual surgical volumes were calculated for each facility to stratify them into quartiles based on highest to lowest case volumes. A multivariable negative binomial mixed model was used to model the number of nodes examined at the patient level, which accommodated the clustering of hospitals and adjusted for clinical and demographic factors.

Results: The median lymph node yield for all hospitals was 27 (Interquartile Range [IQR] 16-40) nodes. Median lymph nodes examined for the quartile of highest volume hospitals was 29 (IQR 19-42). Compared to a lymph node yield of 18 (IQR 9-30) in the 3rd quartile, 17 (IQR 9-27) in the 2nd quartile, and 16 (IQR 8-26) in the lowest quartile. A Dwass, Steel, Critchlow-Fligner procedure indicated that the number of nodes were significantly different for all pairwise comparisons except between the lowest two quartiles.

Conclusions: These data suggest that for patients with oral SCC undergoing neck dissections, increase in hospital case volume is associated with higher lymph node yield. Institutions with greater experience and case volumes are more likely to consistently meet the cutoff value of lymph node yield ( 18 lymph nodes) in neck dissection specimens, that are shown to improve survival outcomes.

Purpose: Lymph node yield is an independent prognostic factor for survival outcomes in patients receiving neck dissection for oral squamous cell carcinoma (SCC). This study investigates whether hospital case volume is associated with increased lymph node yield in neck dissection specimens.

Background: Guidelines recommending minimum number of lymph nodes that constitutes an adequate neck dissection have not been established. There is evidence that comprehensive neck dissections with improved lymph node yield ( 18 nodes) are associated with better survival outcomes. Recent studies have proposed this association to be a potential institutional-level quality metric. However, whetherinstitutional experience (reflected by case volume) influences the quality and comprehensiveness of neck dissection (reflected in node yield) remains unknown.

Methods: The National Cancer Database was used to identify 21,150 patients who underwent primary neck dissections for oral SCC diagnosed between 2004 and 2015. Average annual surgical volumes were calculated for each facility to stratify them into quartiles based on highest to lowest case volumes. A multivariable negative binomial mixed model was used to model the number of nodes examined at the patient level, which accommodated the clustering of hospitals and adjusted for clinical and demographic factors.

Results: The median lymph node yield for all hospitals was 27 (Interquartile Range [IQR] 16-40) nodes. Median lymph nodes examined for the quartile of highest volume hospitals was 29 (IQR 19-42). Compared to a lymph node yield of 18 (IQR 9-30) in the 3rd quartile, 17 (IQR 9-27) in the 2nd quartile, and 16 (IQR 8-26) in the lowest quartile. A Dwass, Steel, Critchlow-Fligner procedure indicated that the number of nodes were significantly different for all pairwise comparisons except between the lowest two quartiles.

Conclusions: These data suggest that for patients with oral SCC undergoing neck dissections, increase in hospital case volume is associated with higher lymph node yield. Institutions with greater experience and case volumes are more likely to consistently meet the cutoff value of lymph node yield ( 18 lymph nodes) in neck dissection specimens, that are shown to improve survival outcomes.

Purpose: Lymph node yield is an independent prognostic factor for survival outcomes in patients receiving neck dissection for oral squamous cell carcinoma (SCC). This study investigates whether hospital case volume is associated with increased lymph node yield in neck dissection specimens.

Background: Guidelines recommending minimum number of lymph nodes that constitutes an adequate neck dissection have not been established. There is evidence that comprehensive neck dissections with improved lymph node yield ( 18 nodes) are associated with better survival outcomes. Recent studies have proposed this association to be a potential institutional-level quality metric. However, whetherinstitutional experience (reflected by case volume) influences the quality and comprehensiveness of neck dissection (reflected in node yield) remains unknown.

Methods: The National Cancer Database was used to identify 21,150 patients who underwent primary neck dissections for oral SCC diagnosed between 2004 and 2015. Average annual surgical volumes were calculated for each facility to stratify them into quartiles based on highest to lowest case volumes. A multivariable negative binomial mixed model was used to model the number of nodes examined at the patient level, which accommodated the clustering of hospitals and adjusted for clinical and demographic factors.

Results: The median lymph node yield for all hospitals was 27 (Interquartile Range [IQR] 16-40) nodes. Median lymph nodes examined for the quartile of highest volume hospitals was 29 (IQR 19-42). Compared to a lymph node yield of 18 (IQR 9-30) in the 3rd quartile, 17 (IQR 9-27) in the 2nd quartile, and 16 (IQR 8-26) in the lowest quartile. A Dwass, Steel, Critchlow-Fligner procedure indicated that the number of nodes were significantly different for all pairwise comparisons except between the lowest two quartiles.

Conclusions: These data suggest that for patients with oral SCC undergoing neck dissections, increase in hospital case volume is associated with higher lymph node yield. Institutions with greater experience and case volumes are more likely to consistently meet the cutoff value of lymph node yield ( 18 lymph nodes) in neck dissection specimens, that are shown to improve survival outcomes.

Background: Chondrosarcoma (CHS) is the second most common primary malignant solid bone tumor. Present management of CHS is based largely on case series from individual treatment centers. This study summarizes descriptive epidemiologic and prognostic characteristics associated with CHS patients utilizing the National Cancer Database (NCDB).

Methods: We identified 6,559 patients with CHS of the bone, joints, and/or articular cartilage in the NCDB from 2004-2015. Kaplan-Meier analyses were used to estimate 2- and 5-year survival, and median survival. Survival comparisons were made using log-rank tests.

Results: Median age at diagnosis was 53 years. 53.3% of cases were in males, and 86.4% were in white patients. 28.2% of cases were in lower limb long bones and associated joints. The most common NCDB analytic stage at presentation was stage I (60.5%). 6.4% of patients had known metastases at diagnosis. 86.6%, 7%, and 12.5% of patients received surgery, chemotherapy, and radiation, respectively. Overall probability of 5-year survival was 74.4%. Approximate 5-year survival for NCDB analytic stages I-IV was 86.2%, 53.1%, 52.4%, and 17.4%, respectively. The dedifferentiated subtype of CHS had the poorest 5-year survival (25.4%), while juxtacortical had the highest (93.4%). In 355 patients with high grade CHS, perioperative chemotherapy (33.7% 5-year survival) did not provide improved survival compared to surgery alone (51.2% 5-year survival).

Conclusions: To the best of our knowledge, this is the first NCDB study to evaluate survival by treatment type and stage in CHS, as well as to analyze epidemiologic and prognostic characteristics in all CHS subtypes. This study found CHS was most common in white patients, had a median age at diagnosis of 53 years, and was most frequently found in lower limb long bones and associated joints. The 5-year survival was much worse at stage IV compared to stages I, II, and III, as well as in dedifferentiated and mesenchymal tumor subtypes compared to conventional or other types of CHS. In high grade tumors, perioperative chemotherapy did not improve survival compared to surgery alone.

Background: Chondrosarcoma (CHS) is the second most common primary malignant solid bone tumor. Present management of CHS is based largely on case series from individual treatment centers. This study summarizes descriptive epidemiologic and prognostic characteristics associated with CHS patients utilizing the National Cancer Database (NCDB).

Methods: We identified 6,559 patients with CHS of the bone, joints, and/or articular cartilage in the NCDB from 2004-2015. Kaplan-Meier analyses were used to estimate 2- and 5-year survival, and median survival. Survival comparisons were made using log-rank tests.

Results: Median age at diagnosis was 53 years. 53.3% of cases were in males, and 86.4% were in white patients. 28.2% of cases were in lower limb long bones and associated joints. The most common NCDB analytic stage at presentation was stage I (60.5%). 6.4% of patients had known metastases at diagnosis. 86.6%, 7%, and 12.5% of patients received surgery, chemotherapy, and radiation, respectively. Overall probability of 5-year survival was 74.4%. Approximate 5-year survival for NCDB analytic stages I-IV was 86.2%, 53.1%, 52.4%, and 17.4%, respectively. The dedifferentiated subtype of CHS had the poorest 5-year survival (25.4%), while juxtacortical had the highest (93.4%). In 355 patients with high grade CHS, perioperative chemotherapy (33.7% 5-year survival) did not provide improved survival compared to surgery alone (51.2% 5-year survival).

Conclusions: To the best of our knowledge, this is the first NCDB study to evaluate survival by treatment type and stage in CHS, as well as to analyze epidemiologic and prognostic characteristics in all CHS subtypes. This study found CHS was most common in white patients, had a median age at diagnosis of 53 years, and was most frequently found in lower limb long bones and associated joints. The 5-year survival was much worse at stage IV compared to stages I, II, and III, as well as in dedifferentiated and mesenchymal tumor subtypes compared to conventional or other types of CHS. In high grade tumors, perioperative chemotherapy did not improve survival compared to surgery alone.

Background: Chondrosarcoma (CHS) is the second most common primary malignant solid bone tumor. Present management of CHS is based largely on case series from individual treatment centers. This study summarizes descriptive epidemiologic and prognostic characteristics associated with CHS patients utilizing the National Cancer Database (NCDB).

Methods: We identified 6,559 patients with CHS of the bone, joints, and/or articular cartilage in the NCDB from 2004-2015. Kaplan-Meier analyses were used to estimate 2- and 5-year survival, and median survival. Survival comparisons were made using log-rank tests.

Results: Median age at diagnosis was 53 years. 53.3% of cases were in males, and 86.4% were in white patients. 28.2% of cases were in lower limb long bones and associated joints. The most common NCDB analytic stage at presentation was stage I (60.5%). 6.4% of patients had known metastases at diagnosis. 86.6%, 7%, and 12.5% of patients received surgery, chemotherapy, and radiation, respectively. Overall probability of 5-year survival was 74.4%. Approximate 5-year survival for NCDB analytic stages I-IV was 86.2%, 53.1%, 52.4%, and 17.4%, respectively. The dedifferentiated subtype of CHS had the poorest 5-year survival (25.4%), while juxtacortical had the highest (93.4%). In 355 patients with high grade CHS, perioperative chemotherapy (33.7% 5-year survival) did not provide improved survival compared to surgery alone (51.2% 5-year survival).

Conclusions: To the best of our knowledge, this is the first NCDB study to evaluate survival by treatment type and stage in CHS, as well as to analyze epidemiologic and prognostic characteristics in all CHS subtypes. This study found CHS was most common in white patients, had a median age at diagnosis of 53 years, and was most frequently found in lower limb long bones and associated joints. The 5-year survival was much worse at stage IV compared to stages I, II, and III, as well as in dedifferentiated and mesenchymal tumor subtypes compared to conventional or other types of CHS. In high grade tumors, perioperative chemotherapy did not improve survival compared to surgery alone.

Background: Primary cardiac sarcomas are rare and highly lethal tumors. Current understanding of clinicopathologic characteristics associated with primary cardiac sarcoma is limited to case reports and small case series. To the best of our knowledge, this is the largest study to evaluate clinicopathologic characteristics of primary cardiac sarcoma, and survival benefit of treatment modalities.

Methods: The National Cancer Database was utilized to retrospectively identify 404 patients diagnosed with a single primary cardiac sarcoma between 2004 and 2015. Kaplan-Meier methods were used to estimate survival distribution. Log-rank tests compared survival distributions between groups.

Results: Median age at diagnosis was 50 years. Men (50.7%) and women (49.3%) were affected equally. The most common histology types were hemangiosarcoma (40.1%), sarcoma NOS (14.9%), spindle cell sarcoma (6.9%) and leiomyosarcoma (5.9%). The most common stage at presentation was stage IV (38.1%). 37% of patients presented with metastases at the time of diagnosis, and the most common site of distant metastasis was the
lung (12.6%). Median overall survival (OS) for the entire cohort was 12 months. Median OS for stages I-IV was 23.06, 22.34, 12.98 and 8.08 months, respectively. 63% of patients underwent surgical resection. 58.4% received chemotherapy, and 22% received radiotherapy. Patients who underwent surgery had improved OS compared to those not receiving surgery (15.74 vs. 7.62 months, respectively). In patients with metastatic disease, OS was improved when treated with chemotherapy (11.14 months), compared to surgery alone (5.22 months). Combined treatment of surgery with chemotherapy did not improve outcomes compared to chemotherapy alone (P = .091 on log-rank test). In non-metastatic disease,
patients receiving multi-modality treatment (radiotherapy, chemotherapy and surgery) had improved OS (27.73 months) compared to those who underwent surgery alone, chemotherapy alone or chemotherapy with surgery (6.67, 14.88 and 20.99 months, respectively).

Conclusions: The median age of diagnosis was 50 years, and men and women are affected equally. Stage IV is the most common stage at diagnosis, and prognosis of Stages III and IV are worse in comparison to Stages I and II. In metastatic disease, receipt of chemotherapy is associated with improved survivorship. Multimodality treatment regimens may be recommended in non-metastatic cases.

Background: Primary cardiac sarcomas are rare and highly lethal tumors. Current understanding of clinicopathologic characteristics associated with primary cardiac sarcoma is limited to case reports and small case series. To the best of our knowledge, this is the largest study to evaluate clinicopathologic characteristics of primary cardiac sarcoma, and survival benefit of treatment modalities.

Methods: The National Cancer Database was utilized to retrospectively identify 404 patients diagnosed with a single primary cardiac sarcoma between 2004 and 2015. Kaplan-Meier methods were used to estimate survival distribution. Log-rank tests compared survival distributions between groups.

Results: Median age at diagnosis was 50 years. Men (50.7%) and women (49.3%) were affected equally. The most common histology types were hemangiosarcoma (40.1%), sarcoma NOS (14.9%), spindle cell sarcoma (6.9%) and leiomyosarcoma (5.9%). The most common stage at presentation was stage IV (38.1%). 37% of patients presented with metastases at the time of diagnosis, and the most common site of distant metastasis was the
lung (12.6%). Median overall survival (OS) for the entire cohort was 12 months. Median OS for stages I-IV was 23.06, 22.34, 12.98 and 8.08 months, respectively. 63% of patients underwent surgical resection. 58.4% received chemotherapy, and 22% received radiotherapy. Patients who underwent surgery had improved OS compared to those not receiving surgery (15.74 vs. 7.62 months, respectively). In patients with metastatic disease, OS was improved when treated with chemotherapy (11.14 months), compared to surgery alone (5.22 months). Combined treatment of surgery with chemotherapy did not improve outcomes compared to chemotherapy alone (P = .091 on log-rank test). In non-metastatic disease,
patients receiving multi-modality treatment (radiotherapy, chemotherapy and surgery) had improved OS (27.73 months) compared to those who underwent surgery alone, chemotherapy alone or chemotherapy with surgery (6.67, 14.88 and 20.99 months, respectively).

Conclusions: The median age of diagnosis was 50 years, and men and women are affected equally. Stage IV is the most common stage at diagnosis, and prognosis of Stages III and IV are worse in comparison to Stages I and II. In metastatic disease, receipt of chemotherapy is associated with improved survivorship. Multimodality treatment regimens may be recommended in non-metastatic cases.

Background: Primary cardiac sarcomas are rare and highly lethal tumors. Current understanding of clinicopathologic characteristics associated with primary cardiac sarcoma is limited to case reports and small case series. To the best of our knowledge, this is the largest study to evaluate clinicopathologic characteristics of primary cardiac sarcoma, and survival benefit of treatment modalities.

Methods: The National Cancer Database was utilized to retrospectively identify 404 patients diagnosed with a single primary cardiac sarcoma between 2004 and 2015. Kaplan-Meier methods were used to estimate survival distribution. Log-rank tests compared survival distributions between groups.

Results: Median age at diagnosis was 50 years. Men (50.7%) and women (49.3%) were affected equally. The most common histology types were hemangiosarcoma (40.1%), sarcoma NOS (14.9%), spindle cell sarcoma (6.9%) and leiomyosarcoma (5.9%). The most common stage at presentation was stage IV (38.1%). 37% of patients presented with metastases at the time of diagnosis, and the most common site of distant metastasis was the
lung (12.6%). Median overall survival (OS) for the entire cohort was 12 months. Median OS for stages I-IV was 23.06, 22.34, 12.98 and 8.08 months, respectively. 63% of patients underwent surgical resection. 58.4% received chemotherapy, and 22% received radiotherapy. Patients who underwent surgery had improved OS compared to those not receiving surgery (15.74 vs. 7.62 months, respectively). In patients with metastatic disease, OS was improved when treated with chemotherapy (11.14 months), compared to surgery alone (5.22 months). Combined treatment of surgery with chemotherapy did not improve outcomes compared to chemotherapy alone (P = .091 on log-rank test). In non-metastatic disease,
patients receiving multi-modality treatment (radiotherapy, chemotherapy and surgery) had improved OS (27.73 months) compared to those who underwent surgery alone, chemotherapy alone or chemotherapy with surgery (6.67, 14.88 and 20.99 months, respectively).

Conclusions: The median age of diagnosis was 50 years, and men and women are affected equally. Stage IV is the most common stage at diagnosis, and prognosis of Stages III and IV are worse in comparison to Stages I and II. In metastatic disease, receipt of chemotherapy is associated with improved survivorship. Multimodality treatment regimens may be recommended in non-metastatic cases.

Background: Prostatic leiomyosarcoma is a rare tumor that accounts for less than 0.1% of all primary prostatic malignancies. This neoplasm is composed of highly aggressive prostatic smooth muscle cells that presents with nonspecific signs and symptoms mimicking other forms of prostatic pathology.

Case Report: A 72-year-old male presented with 6 months of progressive severe lower urinary tract symptoms (LUTS) secondary to bladder outlet obstruction. The patient was refractory to medical management and required multiple emergent bladder catheterizations. Workup with urinalysis, blood biochemistry, and PSA levels were persistently normal. He denied hematuria, weight loss, or perineal pain. On rectal exam, a mass like induration was noted along the right upper lobe of the prostate.

The patient was referred for transurethral resection of the prostate (TURP) for suspected severe BPH. Histopathological examination demonstrated atypical cytology consistent with high-grade leiomyosarcoma. A PET scan revealed a 1.9 cm tumor with uptake of 12.6 SUV in the right posterior prostate.

Discussion: Fewer than 200 cases of prostatic leiomyosarcoma have been reported. Patients typically present between 41-78 years of age (mean age of 61 years) with signs and symptoms related to LUTS (89.4%) and perineal or rectal pain (25.6%). Constitutional symptoms, burning on ejaculation, and hematuria are uncommon. PSA levels remain normal, due to its non-epithelial etiology, which may contribute to delays in diagnosis.

Diagnosis is usually achieved after a TURP procedure or transrectal needle biopsy. Pathology demonstrates neoplastic spindle cells with nuclear atypia, multifocal necrosis, and cystic degeneration. Immunohistochemistry is characteristically positive for desmin, smooth muscle actin, and vimentin. Tumors have been reported to range between 2-31 cm in size, frequently presenting with invasion of local structures. The lungs are the most common site of distant metastasis.

Treatment may include a multimodal approach of surgery, radiation, and chemotherapy. Long-term prognosis is poor due to the tumors aggressive nature, and recurrence is common. Median survival is estimated at 17 months and 50-75% of patients die within 2-5 years of diagnosis. Due to the substantially high risk of death, prostatic leiomyosarcoma may be one of the most aggressive and poorly prognostic malignancies involving the prostate.

Background: Prostatic leiomyosarcoma is a rare tumor that accounts for less than 0.1% of all primary prostatic malignancies. This neoplasm is composed of highly aggressive prostatic smooth muscle cells that presents with nonspecific signs and symptoms mimicking other forms of prostatic pathology.

Case Report: A 72-year-old male presented with 6 months of progressive severe lower urinary tract symptoms (LUTS) secondary to bladder outlet obstruction. The patient was refractory to medical management and required multiple emergent bladder catheterizations. Workup with urinalysis, blood biochemistry, and PSA levels were persistently normal. He denied hematuria, weight loss, or perineal pain. On rectal exam, a mass like induration was noted along the right upper lobe of the prostate.

The patient was referred for transurethral resection of the prostate (TURP) for suspected severe BPH. Histopathological examination demonstrated atypical cytology consistent with high-grade leiomyosarcoma. A PET scan revealed a 1.9 cm tumor with uptake of 12.6 SUV in the right posterior prostate.

Discussion: Fewer than 200 cases of prostatic leiomyosarcoma have been reported. Patients typically present between 41-78 years of age (mean age of 61 years) with signs and symptoms related to LUTS (89.4%) and perineal or rectal pain (25.6%). Constitutional symptoms, burning on ejaculation, and hematuria are uncommon. PSA levels remain normal, due to its non-epithelial etiology, which may contribute to delays in diagnosis.

Diagnosis is usually achieved after a TURP procedure or transrectal needle biopsy. Pathology demonstrates neoplastic spindle cells with nuclear atypia, multifocal necrosis, and cystic degeneration. Immunohistochemistry is characteristically positive for desmin, smooth muscle actin, and vimentin. Tumors have been reported to range between 2-31 cm in size, frequently presenting with invasion of local structures. The lungs are the most common site of distant metastasis.

Treatment may include a multimodal approach of surgery, radiation, and chemotherapy. Long-term prognosis is poor due to the tumors aggressive nature, and recurrence is common. Median survival is estimated at 17 months and 50-75% of patients die within 2-5 years of diagnosis. Due to the substantially high risk of death, prostatic leiomyosarcoma may be one of the most aggressive and poorly prognostic malignancies involving the prostate.

Background: Prostatic leiomyosarcoma is a rare tumor that accounts for less than 0.1% of all primary prostatic malignancies. This neoplasm is composed of highly aggressive prostatic smooth muscle cells that presents with nonspecific signs and symptoms mimicking other forms of prostatic pathology.

Case Report: A 72-year-old male presented with 6 months of progressive severe lower urinary tract symptoms (LUTS) secondary to bladder outlet obstruction. The patient was refractory to medical management and required multiple emergent bladder catheterizations. Workup with urinalysis, blood biochemistry, and PSA levels were persistently normal. He denied hematuria, weight loss, or perineal pain. On rectal exam, a mass like induration was noted along the right upper lobe of the prostate.

The patient was referred for transurethral resection of the prostate (TURP) for suspected severe BPH. Histopathological examination demonstrated atypical cytology consistent with high-grade leiomyosarcoma. A PET scan revealed a 1.9 cm tumor with uptake of 12.6 SUV in the right posterior prostate.

Discussion: Fewer than 200 cases of prostatic leiomyosarcoma have been reported. Patients typically present between 41-78 years of age (mean age of 61 years) with signs and symptoms related to LUTS (89.4%) and perineal or rectal pain (25.6%). Constitutional symptoms, burning on ejaculation, and hematuria are uncommon. PSA levels remain normal, due to its non-epithelial etiology, which may contribute to delays in diagnosis.

Diagnosis is usually achieved after a TURP procedure or transrectal needle biopsy. Pathology demonstrates neoplastic spindle cells with nuclear atypia, multifocal necrosis, and cystic degeneration. Immunohistochemistry is characteristically positive for desmin, smooth muscle actin, and vimentin. Tumors have been reported to range between 2-31 cm in size, frequently presenting with invasion of local structures. The lungs are the most common site of distant metastasis.

Treatment may include a multimodal approach of surgery, radiation, and chemotherapy. Long-term prognosis is poor due to the tumors aggressive nature, and recurrence is common. Median survival is estimated at 17 months and 50-75% of patients die within 2-5 years of diagnosis. Due to the substantially high risk of death, prostatic leiomyosarcoma may be one of the most aggressive and poorly prognostic malignancies involving the prostate.

Background: Dedifferentiated liposarcoma (DDL) is a high-grade tumor that either forms as a de novo tumor or as a progression from a low-grade, well-differentiated liposarcoma. DDL is a clinically aggressive tumor associated with low survival probabilities. Prognosis variables for DDL have never been reported in a large patient population.

Methods: 3,573 patients with primary DDL were analyzed from the National Cancer Database (NCDB). The 5- and 10-year survival probabilities  were calculated, and the groups were compared using log-rank comparisons. Median survival was also calculated.

Results: Males comprised 65% of the cohort, 65.4% of the patients were between the age of 51-75 years old, and the median age at diagnosis was 64 years of age. Approximately 65% of tumors were > 10 cm, 89% had no metastases and the most common site of metastasis was the lung (2.5%). The most common tumors were found in the retroperitoneum or peritoneum (40.5%). Overall 5- and 10-year survival probabilities were 51.5% and 34.8%, respectively. Head and neck tumors had the best 5-year outcomes (86.4%) followed by extremities (67.1%), pelvis (65.8%), thorax or trunk (58.9%), retroperitoneum or peritoneum (45.3%), and abdomen (36.8%). Best outcomes were noted in the 26-50 year age group (5-year survival, 66.8%; 10-year survival, 52.9%), < 1 0 cm diameter size (5-year survival: 66.1%; 10-year survival, 46%), stage II (5-year survival, 66.7%), and moderately differentiated tumors (5-year survival, 68.3%). Radiation therapy yielded the best 5- and 10-year survival probabilities of 59% and 39.3%, respectively. Out of all the adjuvant therapies, surgery with radiation resulted in the best 5-year survival of 63.4%.

Conclusions: In the largest and most comprehensive study to date on DDL, major findings include that primary anatomical site is a significant prognostic variable and age at presentation, tumor size, stage, grade, and type of adjuvant therapy significantly affect median survival and overall survival probabilities.

Background: Dedifferentiated liposarcoma (DDL) is a high-grade tumor that either forms as a de novo tumor or as a progression from a low-grade, well-differentiated liposarcoma. DDL is a clinically aggressive tumor associated with low survival probabilities. Prognosis variables for DDL have never been reported in a large patient population.

Methods: 3,573 patients with primary DDL were analyzed from the National Cancer Database (NCDB). The 5- and 10-year survival probabilities  were calculated, and the groups were compared using log-rank comparisons. Median survival was also calculated.

Results: Males comprised 65% of the cohort, 65.4% of the patients were between the age of 51-75 years old, and the median age at diagnosis was 64 years of age. Approximately 65% of tumors were > 10 cm, 89% had no metastases and the most common site of metastasis was the lung (2.5%). The most common tumors were found in the retroperitoneum or peritoneum (40.5%). Overall 5- and 10-year survival probabilities were 51.5% and 34.8%, respectively. Head and neck tumors had the best 5-year outcomes (86.4%) followed by extremities (67.1%), pelvis (65.8%), thorax or trunk (58.9%), retroperitoneum or peritoneum (45.3%), and abdomen (36.8%). Best outcomes were noted in the 26-50 year age group (5-year survival, 66.8%; 10-year survival, 52.9%), < 1 0 cm diameter size (5-year survival: 66.1%; 10-year survival, 46%), stage II (5-year survival, 66.7%), and moderately differentiated tumors (5-year survival, 68.3%). Radiation therapy yielded the best 5- and 10-year survival probabilities of 59% and 39.3%, respectively. Out of all the adjuvant therapies, surgery with radiation resulted in the best 5-year survival of 63.4%.

Conclusions: In the largest and most comprehensive study to date on DDL, major findings include that primary anatomical site is a significant prognostic variable and age at presentation, tumor size, stage, grade, and type of adjuvant therapy significantly affect median survival and overall survival probabilities.

Background: Dedifferentiated liposarcoma (DDL) is a high-grade tumor that either forms as a de novo tumor or as a progression from a low-grade, well-differentiated liposarcoma. DDL is a clinically aggressive tumor associated with low survival probabilities. Prognosis variables for DDL have never been reported in a large patient population.

Methods: 3,573 patients with primary DDL were analyzed from the National Cancer Database (NCDB). The 5- and 10-year survival probabilities  were calculated, and the groups were compared using log-rank comparisons. Median survival was also calculated.

Results: Males comprised 65% of the cohort, 65.4% of the patients were between the age of 51-75 years old, and the median age at diagnosis was 64 years of age. Approximately 65% of tumors were > 10 cm, 89% had no metastases and the most common site of metastasis was the lung (2.5%). The most common tumors were found in the retroperitoneum or peritoneum (40.5%). Overall 5- and 10-year survival probabilities were 51.5% and 34.8%, respectively. Head and neck tumors had the best 5-year outcomes (86.4%) followed by extremities (67.1%), pelvis (65.8%), thorax or trunk (58.9%), retroperitoneum or peritoneum (45.3%), and abdomen (36.8%). Best outcomes were noted in the 26-50 year age group (5-year survival, 66.8%; 10-year survival, 52.9%), < 1 0 cm diameter size (5-year survival: 66.1%; 10-year survival, 46%), stage II (5-year survival, 66.7%), and moderately differentiated tumors (5-year survival, 68.3%). Radiation therapy yielded the best 5- and 10-year survival probabilities of 59% and 39.3%, respectively. Out of all the adjuvant therapies, surgery with radiation resulted in the best 5-year survival of 63.4%.

Conclusions: In the largest and most comprehensive study to date on DDL, major findings include that primary anatomical site is a significant prognostic variable and age at presentation, tumor size, stage, grade, and type of adjuvant therapy significantly affect median survival and overall survival probabilities.