Confirmed angiosarcoma: prognostic factors and outcome in 50 prospectively followed patients

Purpose. Angiosarcoma is a rare tumor with endothelial cell differentiation that may arise in any anatomic location.The purpose of this report was to identify prognostic factors on outcome in a group of prospectively followed patients with confirmed angiosarcoma.Subjects. Adult patients (>16 years old) with angiosarcoma treated between July 1982 and February 1998 were identified from a prospective database.Methods. Pathologic confirmation of all cases was performed prior to inclusion in this analysis. Various prognostic factors were evaluated for disease-specific survival. Survival was determined by the Kaplan- Meier method. Statistical significance was evaluated by log-rank test for univariate analysis and Cox stepwise regression for multivariate analysis (p<0.05).Results. Fifty patients were identified; at the initial evaluation, this group included 32 patients with a primary tumor, three with local recurrence and 15 with metastatic disease. Tumor sites included 16 head and neck and skin of head, eight extremity, seven trunk, six breast, five pelvis, four viscera and four thoracic. Median follow-up among survivors was 71 months (range, 38-191 months).Two- and 5-year disease-specific survival was 50 and 30%, respectively, with a median of 24 months. The factor predictive of tumor-related mortality was presentation status (p=0.001; relative risk, 5). Two-year disease-specific survival for patients presenting with recurrent or metastatic disease was 13%, compared with 70% for those with primary disease.