Grobman L R, Fisch U, Pollack A
University of Miami School of Medicine, Florida.
Am J Otol. 1990 Mar;11(2):108-12.
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.
中枢神经纤维瘤病是一种神经嵴组织衍生物的遗传性疾病，包括双侧听神经瘤和其他中枢神经系统肿瘤，通常为脑膜瘤或胶质瘤。这与周围神经纤维瘤病不同，因为其主要表现为中枢神经系统症状，且常常缺乏伴随的周围神经纤维瘤和咖啡斑，同时神经生长因子也有不同改变。我们展示了一例未被怀疑的中枢神经纤维瘤病的颞骨组织病理学情况，该病例包括双侧侵犯耳蜗的听神经瘤和一例无症状的多形性胶质母细胞瘤，患者无家族病史且无任何周围神经纤维瘤病的体征。由于双侧肿瘤常侵犯耳蜗，建议采用改良经耳入路而非经迷路入路以完全切除肿瘤。在仍有有用听力时，对侧肿瘤切除应推迟。
