Outcome of locally recurrent and metastatic angiosarcoma

Background: Angiosarcoma (AS) is a rare soft tissue sarcoma with an enhanced propensity for local and systemic failure. The outcome of locally recurrent and metastatic AS treated at a single institution was evaluated. Methods: Medical records of AS patients treated for local recurrence and distant metastasis (1993-2008) were retrospectively reviewed. Univariable and multivariable analyses were performed to identify prognosticators. Results: Forty-four patients were treated for locally recurrent AS; the majority (59%) were ≤5 cm; the most common sites were skin (48%) and breast (32%). Thirty-two patients (73%) had surgery; 73% received chemotherapy; radiation was delivered to 41%. Median disease-specific survival (DSS) was 50 months [95% confidence interval (CI): 25.7-73.5 months]. Multivariable analysis identified size >5 cm as the only independent adverse prognosticator of recurrent AS-specific survival [hazard ratio (HR): 3.26, P = 0.04]. Ninety-nine patients were treated for metastatic AS; 73% had multiple metastatic sites; the lung was the most common site (36%). Chemotherapy, mainly doxorubicin- and/or paclitaxel-based regimens, were administered to 95 patients (96%). Radiotherapy was utilized in 25% cases; 16% of patients underwent curative-intent surgery. Median DSS was 10 months (95% CI: 7.9-12 months). Isolated lymph node metastasis versus hematogenic spread was the only statistically significant favorable prognostic factor identified (HR: 0.29, P = 0.01). Conclusion: Locally recurrent AS is often treatable; complete resection can potentially prolong survival. In contrast, metastatic patients have a grave prognosis; however, patients with isolated lymphatic spread and possibly those treated with taxol-based chemotherapeutic regimens have a favorable outcome.