Clinicopathological analysis of primary cardiac angiosarcoma based on a single clinical center

Objective：To analyze the clinicopathological features of primary cardiac angiosarcoma. Methods：The cardiac malignant tumor cases were collected in Zhongshan Hospital,Fudan University from January 2000 to December 2016. The clinical date, histological characteristics, and immunohistochemical markers were analyzed. The relevant literatures were reviewed. Results：Totally 21 cases of primary cardiac angiosarcoma were collected. Males was more than females （14 vs 7）, the average age was 46.5 years. Most of the tumors originated from the right atrium (15/21) with middle and low differentiation （20/21）. The patients always accompanied with pericardial effusion (17/21）. Histologically, the tumor consisted of spindle cells, vessels cracks, blood cavity and blood lake, and pathological mitotic figure were visible. Ninteen cases of follow-up data were received. The expression percentages of CD34,CD31,FLI-1,F8, and EPG were higher. The average survival time was 11 months, 1-year survival rate was 33.3%, 2-year survival rate was 10.0%. Conclusions：Angiosarcoma always occurs in the right atrium, which always accompanies with pericardial effusion. Evidence of vascular differentiation could be found in histopathology. Angiosarcoma has low to middle differentiation, and expresses a set of immunohistochemical markers. The patients with angiosarcoma have poor prognosis.