Intracerebral neurinomas in a case of von Recklinghausen's disease (neurofibromatosis)
Cruz-Sanchez, F.; Cervos-Navarro, J.; Kashihara, M.; Ferszt, R.
Clinical Neuropathology 6(4): 174-178
1987
ISSN/ISBN: 0722-5091 PMID: 2443294 Document Number: 294752
We report the third case of intracerebral neurinoma in a patient with von Recklinghausen's disease, who died of recurrent subdural hematomas after head injury. A total of ten tumors were found in the brain. Both by histological and ultrastructural standards the multiple tumors resembled neurinomas. The pattern of positivity for S-100 and myelin basic protein indicated the Schwann cell origin of the tumors. The ultrastructural findings indicated Schwann cells and numerous areas showing a well defined basement membrane surrounding spindle-shaped neoplastic cells and other areas lacking basement membrane with Schwann cells embedded in collagen. The present case may be categorized as a mixed form of peripheral and central neurofibromatosis. It seems safe to assume that perivascular nerves are the site of origin of the tumors.