Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease: implications for their natural history, diagnosis, and treatment
Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease: implications for their natural history, diagnosis, and treatment
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DOI：
10.3171/jns.2005.102.3.0503
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发表时间：
2005-03-01
影响因子：
4.1
通讯作者：
Lonser, RR
中科院分区：
文献类型：
--
作者：
Kim, HJ;Butman, JA;Lonser, RR
对象。内淋巴囊肿瘤（ELSTs）通常与von Hippel-Lindau （VHL）病相关，可导致不可逆的听力损失和前庭病变。临床和影像学监测方案为ELSTs的自然史、症状形成机制和治疗适应症提供了新的见解。为了澄清与ELSTs的病理生理和治疗相关的不确定性，作者描述了一系列VHL患者，他们的一系列检查记录了ELSTs的发展。如果连续的临床和影像学研究捕捉到了ELSTs的发展，并且患者接受了肿瘤切除术，则VHL患者被纳入研究。分析患者的临床、听力学、影像学特征及手术结果。本研究纳入连续5例患者（3男2女），平均手术年龄34.8岁，随访6 ~ 18个月。3例患者在神经影像学上发现明显肿瘤前出现了听前庭症状。三名患者的听力丧失与甲状腺素内出血的影像学证据一致。通过迷路后后方石油切开术（RLPP）成功切除了ELSTs。所有患者术后听力稳定，前庭症状消失。无患者复发。VHL患者的听前庭症状，包括听力损失，可能是显微镜下elst的结果。一旦发现ELST，可以通过RLPP完全切除，保留听力并改善前庭症状。在听力仍然存在的情况下，早期发现和手术治疗小型ELSTs，应减少与这些肿瘤相关的听力损失、耳鸣、眩晕和脑神经功能障碍的发生率和严重程度。
Object. Endolymphatic sac tumors (ELSTs), which often are associated with von Hippel-Lindau (VHL) disease, cause irreversible hearing loss and vestibulopathy. Clinical and imaging surveillance protocols provide new insights into the natural history, mechanisms of symptom formation, and indications for the treatment of ELSTs. To clarify the uncertainties associated with the pathophysiology and treatment of ELSTs, the authors describe a series of patients with VHL disease in whom serial examinations recorded the development of ELSTs.Methods. Patients with VHL disease were included if serial clinical and imaging studies captured the development of ELSTs, and the patients underwent tumor resection. The patients' clinical, audiological, and imaging characteristics as well as their operative results were analyzed.Five consecutive patients (three men and two women) with a mean age at surgery of 34.8 years and a follow-up period of 6 to 18 months were included in this study. Audiovestibular symptoms were present in three patients before a tumor was evident on neuroimaging. Imaging evidence of an intralabyrinthine hemorrhage coincided with a loss of hearing in three patients. Successful resection of the ELSTs was accomplished by performing a retrolabyrinthine posterior petrosectomy (RLPP). Hearing stabilized and vestibular symptoms resolved after surgery in all patients. No patient has experienced a recurrence.Conclusions. Audiovestibular symptoms, including hearing loss, in patients with,VHL disease can be the result of microscopic ELSTs. Once an ELST has been detected, it can be completely resected via an RLPP with preservation of hearing and amelioration of vestibular symptoms. Early detection and surgical treatment of small ELSTs, when hearing is still present, should reduce the incidence and severity of hearing loss, tinnitus, vertigo, and cranial nerve dysfunction, which are associated with these tumors.