Surgical resection of endolymphatic sac tumors in von Hippel-Lindau disease: findings, results, and indications.
Surgical resection of endolymphatic sac tumors in von Hippel-Lindau disease: findings, results, and indications.
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von Hippel-Lindau病中内淋巴囊肿瘤的手术切除：发现，结果和适应症。
DOI：
10.1002/lary.23646
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发表时间：
2013-03
期刊：
影响因子：
--
通讯作者：
Lonser RR
中科院分区：
文献类型：
--
作者：
Kim HJ;Hagan M;Butman JA;Baggenstos M;Brewer C;Zalewski C;Linehan WM;Lonser RR
为了明确von Hippel-Lindau（VHL）病相关内淋巴囊肿瘤（ELST）的手术治疗和结局，我们分析了接受ELST切除术的连续VHL患者。回顾性研究了1999年至2010年期间在临床研究中心接受ELST切除术的连续VHL患者。分析一系列临床检查、听力图、影像学检查和手术结果。31例ELST连续患者（15例男性，16例女性）接受了33个肿瘤的切除术（平均随访时间，49.9 ± 48.0个月;范围，1.0-116个月）。1例患者行双侧ELST切除术，1例患者因复发再次手术。手术时的平均年龄为38.2 ± 10.2岁（范围：12-67岁）。29耳（88%）有ELST的直接影像学证据，4耳（12%）没有。平均肿瘤大小为1.3 ± 1.1 cm（范围：0.2-5.2 cm）。2例患者（2耳，6%）无症状，29例患者（31耳，94%）有相关的听前庭症状，包括感音神经性听力损失（28耳，84%）、耳鸣（24耳，73%）和眩晕（21例，68%）。术后，31耳中97%的听力稳定（27）或改善（3）。肿瘤完全切除30耳（91%）。并发症包括2耳（6%）脑脊液漏和1耳（3%）暂时性下颅神经麻痹。ELST的手术切除可以保留听力并减少听前庭功能障碍。早期手术切除可以预防或减少致残性听前庭症状，增加完全切除的机会，并保留听力。
To define the surgical treatment and outcomes of von Hippel-Lindau (VHL) disease-associated endolymphatic sac tumors (ELSTs), we analyzed consecutive VHL patients who underwent ELST resection. Retrospective investigation of consecutive VHL patients who underwent resection of ELSTs at a clinical research center between 1999 and 2010. Analysis of serial clinical examinations, audiograms, imaging studies, and operative findings were analyzed. Thirty-one consecutive patients with ELSTs (15 males, 16 females) underwent resection of 33 tumors (mean follow-up, 49.9 ± 48.0 months; range, 1.0–116 months). One patient had bilateral ELST resections and one patient underwent reoperation for recurrence. Mean age at surgery was 38.2 ± 10.2 years (range, 12–67 years). Whereas 29 ears (88%) had direct radiographic evidence of an ELST, four ears (12%) did not. Mean tumor size was 1.3 ± 1.1 cm (range, 0.2–5.2 cm). Whereas two patients (two ears, 6%) were asymptomatic, 29 patients (31 ears, 94% of ears) had associated audiovestibular symptoms, including sensorineural hearing loss (28 ears, 84%), tinnitus (24 ears,73%), and vertigo (21 patients, 68%). Postoperatively, hearing was stabilized (27) or improved (three) in 97% of 31 ears. Complete tumor resection was achieved in 30 ears (91% of 33 ears). Complications included cerebrospinal fluid leak in two ears (6%) and transient lower cranial nerve palsy in one ear (3%). Surgical resection of ELSTs can be performed with hearing preservation and a reduction in audiovestibular dysfunction. Early surgical resection can prevent or decrease disabling audiovestibular symptoms, enhance the opportunity for complete resection, and preserve hearing.
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影响因子：
2.6
作者：
Megerian, CA;Pilch, BZ;McKenna, MJ
通讯作者：
McKenna, MJ
影响因子：
120.7
作者：
Butman, John A.;Kim, H. Jeffrey;Lonser, Russell R.
通讯作者：
Lonser, Russell R.
影响因子：
11.5
作者：
Kaelin, William G., Jr.
通讯作者：
Kaelin, William G., Jr.
影响因子：
4.1
作者：
Jagannathan, Jay;Butman, John A.;Kim, H. Jeffrey
通讯作者：
Kim, H. Jeffrey
影响因子：
4.1
作者：
Kim, HJ;Butman, JA;Lonser, RR
通讯作者：
Lonser, RR