Angiosarcomas, a heterogeneous group of sarcomas with specific behavior depending on primary site: a retrospective study of 161 cases.

Background: Angiosarcomas are rare, heterogeneous and a retrospective study was conducted to describe their natural history. Patients and methods: We reviewed 161 files of angiosarcoma treated in three institutions of the French Sarcoma Group from 1980 to 2004. Survival and prognostic factors for survival were analyzed. Results: Median age was 52 years. Primary sites were the breast (35%), skin (20%) and soft tissues (13%). At initial diagnosis, 31 (19%) had metastases. Surgery was the first treatment in 121 (75%) patients combined with chemotherapy or radiotherapy in 34 and 32, respectively. Ninety (74%) of these 121 patients relapsed, mostly locally (50). With an average time since initial diagnosis of 8.1 years, 123 (76%) patients progressed and 76 (47%) died. Median survival was 3.4 years [95% confidence interval (CI) 2.4-5.8], and the 5-year overall survival (OS) rate was 43% (95% CI 33-53). In multivariate analysis, liver primary site [relative risk (RR) = 12.621, performance status (PS) of two or more (FIR = 3.83), presence of metastases at diagnosis (RR = 2.50), soft tissue tumor (FIR = 0.31) were correlated to OS. PS, liver and soft tissue tumors were identified as independent prognostic factors for progression-free survival. Conclusions: Angiosarcomas have an overall poor outcome, but with a clearly distinct prognosis depending on the primary site.