Vardell VA

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.