Watanabe Kentaro, Cobb Mary In-Ping Huang, Zomorodi Ali R, Cunningham Calhoun D, Nonaka Yoichi, Satoh Shunsuke, Friedman Allan H, Fukushima Takanori
Department of Neurosurgery, Duke University Medical Center, Durham, North Carolina, USA; Department of Neurosurgery, Hôpital Lariboisirère, Paris VII-Diderot University, Paris, France.
Department of Neurosurgery, Duke University Medical Center, Durham, North Carolina, USA.
World Neurosurg. 2017 Mar;99:200-209. doi: 10.1016/j.wneu.2016.12.003. Epub 2016 Dec 10.
Approximately 95% of tumors occurring within the internal auditory canal (IAC) are vestibular schwannomas. Many undergo stereotactic radiation without definitive tissue diagnosis. Rare IAC tumors are not all radiosensitive and are poorly described.
Between 1992 and 2015, 289 consecutive patients with IAC lesions operated on were reviewed retrospectively.
Fifteen patients (5.2%) (16 operations) had unusual histologic findings, including nonvestibular schwannomas (2 facial schwannomas, 2 cochlear schwannomas, 2 intermedius schwannomas), 3 meningiomas, 3 cavernous hemangiomas, a mucosa-associated lymphoid tissue lymphoma, an arachnoid cyst, and a lipochoristoma. None of these rare tumors could be identified before surgery. Three operative approaches were used: the retrosigmoid approach, middle fossa subtemporal approach, or translabyrinthine approach. Few complications occurred, including facial nerve palsy, loss of hearing, and vestibular function. Five-year average follow-up revealed one patient with recurrence.
Clinical examination and imaging alone were insufficient to correctly identify these tumors. Definitive pathologicdiagnosis should be strongly considered to help tailor treatment.
内耳道（IAC）内发生的肿瘤约95%为前庭神经鞘瘤。许多患者在未获得明确组织诊断的情况下接受了立体定向放射治疗。罕见的IAC肿瘤并非都对放射敏感，且相关描述较少。
回顾性分析1992年至2015年间连续接受IAC病变手术的289例患者。
15例患者（5.2%）（16例手术）有异常组织学表现，包括非前庭神经鞘瘤（2例面神经鞘瘤、2例耳蜗神经鞘瘤、2例中间神经鞘瘤）、3例脑膜瘤、3例海绵状血管瘤、1例黏膜相关淋巴组织淋巴瘤、1例蛛网膜囊肿和1例迷芽瘤。这些罕见肿瘤在手术前均无法确诊。采用了三种手术入路：乙状窦后入路、中颅窝颞下入路或经迷路入路。并发症较少，包括面神经麻痹、听力丧失和前庭功能丧失。平均5年随访发现1例患者复发。
仅靠临床检查和影像学检查不足以正确识别这些肿瘤。应强烈考虑进行明确的病理诊断以指导治疗方案的制定。
