Schittenhelm J
Abteilung Neuropathologie, Department für Pathologie und Neuropathologie und Zentrum für Neuroonkologie, Comprehensive Cancer Center, Universitätsklinikum Tübingen, Eberhard-Karls-Universität Tübingen, Calwerstr. 3, 72076, Tübingen, Deutschland.
Pathologe. 2017 Nov;38(6):521-528. doi: 10.1007/s00292-017-0358-x.
Tumors of the inner ear and adjacent structures often present with hearing loss, tinnitus and vertigo due to compression of the traversing cranial nerves. More than 90% of the tumors of the inner ear with or without expansion into the cerebellopontine angle are histologically diagnosed as vestibular schwannomas. Less common tumorous lesions include ectopic meningiomas located in the petrous bone, glomus tympanicum paragangliomas or endolymphatic sac tumors (ELST) originating in the vestibular recess. Most tumors are sporadic, but hereditary disorders have to be considered. Bilateral vestibular schwannomas are indicative of neurofibromatosis type 2 and ELST in conjunction with other abdominal tumors indicates von Hippel-Lindau disease. The neuropathological diagnostics and grading guides the subsequent therapy of these mostly benign lesions.
内耳及相邻结构的肿瘤常因穿行的颅神经受压而出现听力损失、耳鸣和眩晕。超过90%的内耳肿瘤，无论是否扩展至小脑脑桥角，组织学诊断为前庭神经鞘瘤。较少见的肿瘤性病变包括位于岩骨的异位脑膜瘤、鼓室球副神经节瘤或起源于前庭隐窝的内淋巴囊肿瘤（ELST）。大多数肿瘤为散发性，但必须考虑遗传性疾病。双侧前庭神经鞘瘤提示2型神经纤维瘤病，ELST合并其他腹部肿瘤提示冯·希佩尔-林道病。神经病理学诊断和分级指导这些大多为良性病变的后续治疗。
