Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage - Case report
Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage - Case report
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DOI：
10.3171/jns-07/08/0421
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发表时间：
2007-08-01
影响因子：
4.1
通讯作者：
Kim, H. Jeffrey
中科院分区：
文献类型：
--
作者：
Jagannathan, Jay;Butman, John A.;Kim, H. Jeffrey
内淋巴囊肿瘤（ELST）是发生在后岩骨的局部浸润性肿瘤，与von Hippel-Lindau（VHL）病相关。这些肿瘤即使在显微镜下也会引起症状（低于成像研究的可检测性阈值），并可能导致听力损失，耳鸣，眩晕和面部神经功能障碍等症状。虽然ELST患者的听前庭功能障碍的机制尚不完全清楚，但它们对管理具有重要意义。作者介绍了一个33岁的男性VHL疾病和10年的历史进行性耳鸣，眩晕，左侧听力损失的情况下。系列TI加权磁共振（MR）成像和计算机断层扫描显示没有肿瘤的证据，但听力损失后获得的液体衰减反转恢复（FLAIR）MR成像序列显示左侧脑干内出血的证据。根据进行性致残性听前庭功能障碍（耳鸣和眩晕）、FLAIR成像结果和VHL疾病状态，患者接受了岩后区手术探查，发现并完全切除了小（2 mm）ELST。术后，患者耳鸣和眩晕改善。脑干内出血可能是VHL疾病和听前庭功能障碍患者ELST的早期和唯一的神经影像学体征。这些发现支持肿瘤相关出血是ELST相关听前庭功能障碍的潜在机制。
Endolymphatic sac tumors (ELSTs) are locally invasive neoplasms that arise in the posterior petrous bone and are associated with von Hippel-Lindau (VHL) disease. These tumors cause symptoms even when microscopic in size (below the threshold for detectability on imaging studies) and can lead to symptoms such as hearing loss, tinnitus, vertigo, and facial nerve dysfunction. While the mechanisms of audiovestibular dysfunction in patients harboring ELSTs are incompletely understood, they have critical implications for management. The authors present the case of a 33-year-old man with VHL disease and a 10-year history of progressive tinnitus, vertigo, and left-sided hearing loss. Serial TI weighted magnetic resonance (MR) imaging and computed tomography scans revealed no evidence of tumor, but fluid attenuated inversion recovery (FLAIR) MR imaging sequences obtained after hearing loss demonstrated evidence of left intralabyrinthine hemorrhage. On the basis of progressive disabling audiovestibular dysfunction (tinnitus and vertigo), FLAIR imaging findings, and VHL disease status, the patient underwent surgical exploration of the posterior petrous region, and a small (2-mm) ELST was identified and completely resected. Postoperatively, the patient had improvement of the tinnitus and vertigo. Intralabyrinthine hemorrhage may be an early and the only neuroimaging sign of an ELST in patients with VHL disease and audiovestibular dysfunction. These findings support tumor-associated hemorrhage as a mechanism underlying the audiovestibular dysfunction associated with ELSTs.