Factors associated with overall and cancer specific mortality in primary cardiac sarcoma.

Abstract

e23539 Background: Primary cardiac sarcomas (PCS) are extremely rare malignant tumors involving the heart. Only isolated case reports have been described. There is a paucity of data on the epidemiological characteristics of PCS. Virtually all types of sarcomas have been reported in the heart. For most types, only isolated case reports have been described. We used a nationally representative database to evaluate the independent prognostic factors amongst patients with PCS. This study has the objective of investigating the epidemiologic characteristics, survival outcomes and independent prognostic factors of PCS. Methods: We enrolled a total of 362 patients with PCS, between 2000 and 2017, by retrieving the Surveillance, Epidemiology, and End Results (SEER) database. We analyzed demographics, clinical characteristics, and overall mortality (OM) as well as cancer-specific mortality (CSM) of PCS. Variables with a p value < 0.1 in the univariate Cox regression were incorporated into the multivariate Cox model to determine the independent prognostic factors, with a hazard ratio (HR) of greater than 1 representing adverse prognostic factors.All statistical tests were performed by using Software STATA 16.1. The SEER database is a publicly available dataset for which there was no need for IRB approval. Results: The male gender (50.83%), and non-Hispanic whites (58.84%) were the most represented groups in our cohort. The most encountered histologic subtype was angiosarcoma (42.51%), which represented nearly half the cases. Multivariate cox proportional hazard regression analyses of factors affecting all-cause mortality and PCS related mortality among US patients between 2000 and 2017 demonstrated Higher overall mortality in age 80+ (HR = 13.261, 95% CI 5.839-30.119, p = 0), followed by age 60-79 (HR = 1.916, 95% CI 1.213-3.025, p = 0.005); and advanced disease with distant metastasis (HR = 2.013, 95% CI 1.355-2.99, p = 0.001), followed by regional involvement (HR = 1.518, 95% CI 1.041-2.214, p = 0.03). Lower OM rate was observed in patients with rhabdomyosarcoma (HR = 0.364, 95% CI 0.154-0.86, p = 0.021) and widowed (HR = 0.506, 95% CI 0.263-0.977, p = 0.042). Age 80+ (HR = 11.177, 95% CI 4.449-28.08, p = 0) and advanced disease with distant metastases (HR = 2.117, 95% CI 1.37-3.271, p = 0.001) have the highest cancer specific mortality. Patients with rhabdomyosarcoma (HR = 0.344, 95% CI 0.128-0.929, p = 0.035) have a lower CSM. Conclusions: In this United States population-based retrospective cohort study using the SEER database, we found that age at diagnosis and advanced disease at diagnosis were independent factors predicting poor prognosis. Furthermore, patients with rhabdomyosarcoma had a lower mortality compared to other histologic subtypes. Surgical resection of the primary tumor did not significantly impact the overall mortality or the cancer-specific mortality.