Primary cerebral epithelioid angiosarcoma: a case report

BACKGROUND: Primary cerebral epithelioid angiosarcoma (PCEA) is a rare malignant tumor of the central nervous system. To the best of our knowledge, only three cases have been reported in the English language literature thus far. CASE PRESENTATION: Here, we report a fourth case in a 73-year-old man admitted for headache. Radiological examination revealed a mass in the right occipital lobe, which was removed by right occipital craniotomy. Histopathological examination revealed epithelioid angiosarcoma. The patient received radiotherapy after resection but survived for only nine months due to recurrence of the tumor and his declining further surgery. CONCLUSIONS: In this report, we add to the knowledge base on this exceedingly rare tumor, review the small number of relevant cases published previously, and analyze and summarize the clinical and pathological characteristics, treatment options and prognosis of this cancer.