Surgical resection of endolymphatic sac tumors in von Hippel-Lindau disease: findings, results, and indications.
OBJECTIVE
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.


METHODS
Retrospective investigation of consecutive VHL patients who underwent resection of ELSTs at a clinical research center between 1999 and 2010.


METHODS
Analysis of serial clinical examinations, audiograms, imaging studies, and operative findings were analyzed.


RESULTS
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%).


CONCLUSIONS
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.
Office of the Clinical Director and Otolaryngology Branch, National Institute on Deafness and Other Communication Disorders, Georgetown University Medical Center, Washington, DC, USA. hk7@georgetown.edu
H Jeffrey
HJ
Kim
Marygrace
M
Hagan
John A
JA
Butman
Martin
M
Baggenstos
Carmen
C
Brewer
Christopher
C
Zalewski
W Marston
WM
Linehan
Russell R
RR
Lonser
D000293:Adolescent; D000328:Adult; D000368:Aged; D001299:Audiometry; D002648:Child; D004428:Ear Neoplasms / Q000150:complications* / Q000601:surgery*; D004712:Endolymphatic Sac / Q000473:pathology* / Q000601:surgery*; D005260:Female; D005838:Genotype; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009154:Mutation; D012189:Retrospective Studies; D016896:Treatment Outcome; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000235:genetics