Angiosarcoma arising in schwannoma of cerebellopontine angle and later associating with meningioma in a patient with neurofibromatosis type 2.

Here, we describe an extremely rare case of angiosarcomaarising in schwannomaof the cerebellopontine angleand later associating with meningiomain a patient with neurofibromatosis type 2. A 33-year-old disabled Japanese man with right drop footafter surgery for an unspecified tumor demonstrated multiple tumors, suspected to be schwannoma, in the bilateral cerebellopontine angles, the cervical and lumbar spinal cord, and on the right nuchal skin. Also present were several tumors in the medulla and thoracic spinal cord suspected to be ependymomaor astrocytoma. The patient was diagnosed with neurofibromatosis type 2according to the diagnostic criteria by the U.S. National Institutes of Health. The bilateral tumors in the cerebellopontine anglewere resected to reduce symptoms and brain stem compression. Histopathological analysis revealed angiosarcomaarising in schwannomaof the bilateral tumors, and angiosarcomawas proportionally larger in the right tumor than in the left. At age 36, the patient underwent a second resection of the regrown tumor in the left cerebellopontine angle, and histopathology demonstrated mixed angiosarcomaand meningioma. That angiosarcomaarises in schwannomais a pathogenesis within the realm of conjecture, especially that the phenomenon of mixed meningiomaand angiosarcomahas not been reported to date.