OBJECTIVES/HYPOTHESIS: 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. STUDY DESIGN: 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.
OBJECTIVES/HYPOTHESIS: To define the surgical treatment and outcomes of von Hippel-Lindau (VHL) disease-associated endolymphatic sac tumors (ELSTs), we analyzed consecutive VHLpatients who underwent ELST resection. STUDY DESIGN: Retrospective investigation of consecutive VHLpatients 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.
Authors: H Jeffrey Kim; John A Butman; Carmen Brewer; Christopher Zalewski; Alexander O Vortmeyer; Gladys Glenn; Edward H Oldfield; Russell R Lonser Journal: J Neurosurg Date: 2005-03 Impact factor: 5.115
Authors: E R Maher; L Iselius; J R Yates; M Littler; C Benjamin; R Harris; J Sampson; A Williams; M A Ferguson-Smith; N Morton Journal: J Med Genet Date: 1991-07 Impact factor: 6.318
Authors: Russell R Lonser; Gladys M Glenn; McClellan Walther; Emily Y Chew; Steven K Libutti; W Marston Linehan; Edward H Oldfield Journal: Lancet Date: 2003-06-14 Impact factor: 79.321
Authors: John A Butman; H Jeffrey Kim; Martin Baggenstos; Joshua M Ammerman; James Dambrosia; Athos Patsalides; Nicholas J Patronas; Edward H Oldfield; Russell R Lonser Journal: JAMA Date: 2007-07-04 Impact factor: 56.272
Authors: Gautam U Mehta; H Jeffery Kim; Paul W Gidley; Anthony B Daniels; Mia E Miller; Gregory P Lekovic; John A Butman; Russell R Lonser Journal: J Neurol Surg B Skull Base Date: 2021-04-08
Authors: Julian Künzel; Abbas Agaimy; Joachim Hornung; Michael Lell; Oliver Ganslandt; Sabine Semrau; Johannes Zenk Journal: Int J Clin Exp Pathol Date: 2014-04-15
Authors: Arturo Mario Poletti; Siba Prasad Dubey; Giovanni Colombo; Giovanni Cugini; Antonio Mazzoni Journal: Rep Pract Oncol Radiother Date: 2015-07-30
Authors: Shigeru Kawabata; M Christine Hollander; Jeeva P Munasinghe; Lauren R Brinster; José R Mercado-Matos; Jie Li; Lucia Regales; William Pao; Pasi A Jänne; Kwok-Kin Wong; John A Butman; Russell R Lonser; Marlan R Hansen; Richard K Gurgel; Alexander O Vortmeyer; Phillip A Dennis Journal: Oncotarget Date: 2015-05-10
Authors: Neha Varshney; Amanuel A Kebede; Harry Owusu-Dapaah; Jason Lather; Manu Kaushik; Jasneet S Bhullar Journal: J Kidney Cancer VHL Date: 2017-08-02