Epithelioid angiosarcoma: a clinicopathological study of 16 Chinese cases

Aims: To review retrospectively 16 cases of epithelioid angiosarcomas (EAs) with emphasis on their clinical and pathological characteristics, treatment and possible prognostic factors. Methods and results: All eligible cases were searched and acquired from archives of the pathology departments of two hospitals in Shanghai, The Fifth People’s Hospital of Shanghai, Fudan University, and the Shanghai Cancer Center, Fudan University, China. The patients ranged in age from 19 to 77 years, and 5 patients were below 50 years of age. Microscopically, the tumors were mostly composed of large, round or polygonal epithelioid cells that were predominantly arranged in solid sheets or nests. The tumor cells had basophilic or eosinophilic cytoplasm, vesicular nuclei, and prominent nucleoli. Mitotic figures including abnormal mitoses were frequently encountered. In all 16 cases in our series, immunohistochemical studies showed positivity for CD31, and partial positivity for Fli-1, CD34 and factor VIII-related antigen. Of the 14 patients available for follow-up, 3 patients were alive with disease, 9 patients died as a result of the tumor, 1 died of local hemorrhage, and one died of unknown etiology. The median survival was 17.1 months. Conclusions: EA is highly aggressive and carries a very poor prognosis. Therefore, the clinical recognition and correct diagnosis of EA are essential.