Ermann DA

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.

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: 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.