Primary cerebral epithelioid angiosarcoma: a case report

Abstract

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.

Primary cerebral angiosarcoma: a case report

“…They can be found anywhere in the body, mainly in deep soft tissues. Primary cerebral epithelioid angiosarcoma is extremely rare; four cases have been reported in the literature thus far (Table 1 ) 4 – 7 .…”

“…The etiology of epithelioid cerebral angiosarcoma is still unknown; they are more predominant in males compared to females. Risk factors for the development of angiosarcoma include exposure to vinyl chloride, thorotrast contrast, radiation therapy, chronic lymphedema, and familial syndromes, such as neurofibromatosis type 1 1 , 7 . Radiation exposure is one of the most common risk factors for angiosarcoma.…”