Central neurofibromatosis: a clinical-pathological correlation

Central neurofibromatosis is a genetic disorder of neural crest tissue derivatives that includes bilateral acoustic neuromas and other central nervous system tumors, usually meningiomas or gliomas. This is different from peripheral neurofibromatosis because of the primary central nervous system manifestations and frequent lack of accompanying peripheral neurofibromas and café au lait spots as well as different alterations of nerve growth factor. We present the temporal bone histopathology of a unique case of unsuspected central neurofibromatosis that included bilateral acoustic neuromas invading the cochlea and an asymptomatic glioblastoma multiforme occurring in the absence of a family history and without any accompanying peripheral stigmata of neurofibromatosis. As bilateral tumors frequently invade the cochlea, the modified transotic rather than the translabyrinthine approach is recommended for complete tumor removal. Contralateral tumor removal should be delayed while useful hearing remains.