Angiosarcoma is a rare malignancy, and the data about its clinicopathological features and prognostic factors are limited. The purpose of this study was to present a retrospective analysis of angiosarcoma patients treated at a single institution.
Clinical and pathological features of 41 patients treated in our institution between 1998 and 2016 were analyzed.
Of the 41 patients included, 53.7% were women and 46,3% were men and median age was 60 (16-81). 34.1% cases were located in the soft tissues (ST) of the limbs or trunk, 24.4% in the viscera (V), 19.5% in the breast (B), 14.6% involved the head and neck (HN) and 7.3% in the bone (B). The overall survival (OS) at 5 years was 26% (IC: 15% - 42%), with a median survival of 14.9 months. For the 17.1% patients that were alive at the time of analysis, the median follow up was 60 months (16.1-222.1).The tumor sites for the 7 surivors involved B(4), ST(1), HN(1) and V(1). Radiation induced angiosarcoma was suspected in 17.1% patients. Chronic lymphedema was described/highly probable for 19.5% of the patients. Surgery(S) was performed in 82.9% cases, chemotherapy(CT) in 41.5% cases, and radiation therapy(RT) for 9.8% of cases. CT was more likely to be given to younger patients, but did not improve OS. The pathology report was uncertain for 39% cases at first examination. Superficial tumors (B, ST and HN) had a better OS than deep tumors (V,B) but not statistically significant (31%vs15%). Women had a significant better OS compared to men (36%vs16%, p = 0.02). OS at 5 years for patients under age 67 was 30%, and 17% for patients above 67 (p = 0.07). Tumor grade was assesed for 85.4% cases. Grade 1(G1) was found in 12.2%, G2 in 36.6% and G3 in 36.6% patients. Patients having G3 tumors had a lower OS, but not statistically significant. Relapse occured at 58.5% of the patients (from 1 to 4 episodes). Of all relapses, 68.3% occured locally, 7.3% regionally, and 48.8% were distant metastases. The most common metastatic sites were lung (7 cases), bone (4), skin (4) and liver (3).
Angiosarcoma is an agressive tumor that was often underrecognized, occuring in various sites. Women with history of radiotherapy or lymphedema are at higher risk, but have a better prognosis. Younger patients and lower grade seem to have a longer survival.
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All authors have declared no conflicts of interest.