Angiosarcoma-related cerebral metastases: a systematic review of the literature

Angiosarcoma-related cerebral metastases have only been recorded in a few case reports and case series and have not been systematically reviewed to date. Our objective was therefore to perform a systematic literature review on cases of angiosarcomas metastasizing to the brain to inform current practice. All three major libraries—PubMed/MEDLINE, Embase, and Cochrane—were systematically searched, until January 2019. Articles in English reporting angiosarcoma-related cerebral metastases via hematogenous route were included. Our search yielded 45 articles (38 case reports, 5 retrospective studies, 1 case series and 1 letter to the editor), totaling 48 patients (mean age 47.9 years). The main primary site was the heart. The mean time of diagnosis of cerebral metastases following primary tumor identification was 4.9 months. In 15 cases, the brain was the only metastatic site. In cases of multiple extracerebral metastases, the most common sites were the lung and bone. Acute intracerebral supratentorial hemorrhage was the most common presenting radiological feature. Treatment strategies were almost equally divided between the surgical (with or without adjuvant treatment) and the medical arm. Mean overall survival was 7.2 months while progression-free survival was 1.5 months. To our knowledge, this is the first systematic literature review on angiosarcoma-related cerebral metastases. This pathology proves to be an extremely rare clinical entity and carries a poor prognosis, and no consensus has been reached regarding treatment.