内淋巴囊肿瘤的诊治现状和进展

Current opinions on diagnosis and management of endolymphatic sac tumors

内淋巴囊肿瘤(ELST)是一种罕见的、起源于内淋巴囊或内淋巴管的低级别神经外胚层肿瘤,生长缓慢,呈浸润性破坏颞骨,可累及内耳、面神经、颈静脉孔甚至颅内,导致听力下降、眩晕、耳鸣、面瘫等。ELST可散发或是冯希佩尔-林道综合征(VHL综合征)的一部分,其治疗首选手术切除,术式根据肿瘤的临床分级而定。术前常规进行电测听、颞骨高分辨率CT、颞骨增强磁共振成像(MRI)、内耳MRI造影检查,累及血管者术前还应行磁共振动/静脉成像。疑似VHL综合征的患者术前应行基因检测、眼底及腹部超声检查或正电子发射计算机断层显像(PET-CT)。ELST有局部复发的可能,因此术后应定期随访。本文结合我们诊治ELST的临床经验,介绍ELST的诊断、临床分级、术式选择及预后新进展。

Endolymphatic sac tumors(ELST) are rare, slow-growing, low-grade neuroectodermal tumors arising from the endolymphatic sac. ELST can be locally aggressive, infiltrating the petrous bone,resulting in hearing loss, tinnitus, facial palsy and vertigo. In addition, ELST can be sporadic or part of von Hippel-Lindau syndrome(VHL syndrome). The optimal management for ELST is surgical intervention. And the appropriate surgical approach is determined according to the tumor classification. Preoperative routine examination should include pure tone audiometry, high-resolution temporal bone CT scan, enhanced temporal bone magnetic resonance imaging(MRI) and endolymphatic hydrops visualization by inner ear MRI contrast examination. For the patients with potential blood vessel involvement, magnetic resonance arteriography/venography(MRA/MRV) examination are required. Genetic testing, fundus and abdominal ultrasound or positron emission tomography-CT(PET-CT) should be conducted in suspected VHL patients. Given the tendency to recurrence, ELST should be regularly followed up postoperatively. Combined with our clinical practice, we summarized the current opinions on diagnosis, grading, treatment and prognosis of ELST.