Angiosarcomas rarely arise from schwannomas, but we describe here a case of angiosarcoma that arose from a remnant of a benign vestibular schwannoma that had been removed 10 years earlier. The patient was a 66-year-old man with no sign of neurofibromatosis. Although we attempted surgical resection, we could not totally remove the tumour. The patient died nine months after diagnosis, primarily as result of an abscess in the cerebellum and base of the skull. The histological diagnosis was confirmed by the immunohistochemical findings of positivity for CD34 antigen and S-100 protein in the resected tumour.

A review of the literature revealed four other cases of angiosarcoma with schwannoma, all of which arose from an extracranial nerve. The present case is the first report of an angiosarcoma with schwannoma arising from an intracranial locus.