OBJECT: Although endolymphatic sac tumors (ELSTs) frequently destroy the posterior petrous bone and cause hearing loss, the anatomical origin of these neoplasms is unknown. To determine the precise topographic origin of ELSTs, the authors analyzed the imaging, operative, and pathological findings in patients with von Hippel-Lindau disease (VHL) and ELSTs. METHODS: Consecutive VHL patients with small (<or= 1.5 cm) ELSTs who underwent resection at the National Institutes of Health were included. Clinical, imaging, operative, and pathological findings were analyzed. RESULTS: Ten consecutive VHL patients (6 male and 4 female) with 10 small ELSTs (<or= 1.5 cm; 9 left, 1 right) were included. Serial imaging captured the development of 6 ELSTs and revealed that they originated within the intraosseous (vestibular aqueduct) portion of the endolymphatic duct/sac system. Imaging just before surgery demonstrated that the epicenters of 9 ELSTs (1 ELST was not visible on preoperative imaging) were in the vestibular aqueduct. Inspection during surgery established that all 10 ELSTs were limited to the intraosseous endolymphatic duct/sac and the immediately surrounding region. Histological analysis confirmed tumor within the intraosseous portion (vestibular aqueduct) of the endolymphatic duct/sac in all 10 patients. CONCLUSIONS: ELSTs originate from endolymphatic epithelium within the vestibular aqueduct. High-resolution imaging through the region of the vestibular aqueduct is essential for diagnosis. Surgical exploration of the endolymphatic duct and sac is required for complete resection.
OBJECT: Although endolymphatic sac tumors (ELSTs) frequently destroy the posterior petrous bone and cause hearing loss, the anatomical origin of these neoplasms is unknown. To determine the precise topographic origin of ELSTs, the authors analyzed the imaging, operative, and pathological findings in patients with von Hippel-Lindau disease (VHL) and ELSTs. METHODS: Consecutive VHLpatients with small (<or= 1.5 cm) ELSTs who underwent resection at the National Institutes of Health were included. Clinical, imaging, operative, and pathological findings were analyzed. RESULTS: Ten consecutive VHLpatients (6 male and 4 female) with 10 small ELSTs (<or= 1.5 cm; 9 left, 1 right) were included. Serial imaging captured the development of 6 ELSTs and revealed that they originated within the intraosseous (vestibular aqueduct) portion of the endolymphatic duct/sac system. Imaging just before surgery demonstrated that the epicenters of 9 ELSTs (1 ELST was not visible on preoperative imaging) were in the vestibular aqueduct. Inspection during surgery established that all 10 ELSTs were limited to the intraosseous endolymphatic duct/sac and the immediately surrounding region. Histological analysis confirmed tumor within the intraosseous portion (vestibular aqueduct) of the endolymphatic duct/sac in all 10 patients. CONCLUSIONS: ELSTs originate from endolymphatic epithelium within the vestibular aqueduct. High-resolution imaging through the region of the vestibular aqueduct is essential for diagnosis. Surgical exploration of the endolymphatic duct and sac is required for complete resection.
Authors: Russell R Lonser; H Jeffrey Kim; John A Butman; Alexander O Vortmeyer; Daniel I Choo; Edward H Oldfield Journal: N Engl J Med Date: 2004-06-10 Impact factor: 91.245
Authors: Sven Gläsker; Russell R Lonser; Maxine G B Tran; Barbara Ikejiri; John A Butman; Weifen Zeng; Patrick H Maxwell; Zhengping Zhuang; Edward H Oldfield; Alexander O Vortmeyer Journal: Cancer Res Date: 2005-12-01 Impact factor: 12.701
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: T J Manski; D K Heffner; G M Glenn; N J Patronas; A T Pikus; D Katz; R Lebovics; K Sledjeski; P L Choyke; B Zbar; W M Linehan; E H Oldfield Journal: JAMA Date: 1997-05-14 Impact factor: 56.272
Authors: Cliff A Megerian; David S Haynes; Dennis S Poe; Daniel I Choo; Thomas J Keriakas; Michael E Glasscock Journal: Otol Neurotol Date: 2002-05 Impact factor: 2.311
Authors: A O Vortmeyer; M G B Tran; W Zeng; S Gläsker; C Riley; M Tsokos; B Ikejiri; M J Merrill; M Raffeld; Z Zhuang; R R Lonser; P H Maxwell; E H Oldfield Journal: J Pathol Date: 2006-11 Impact factor: 7.996
Authors: W W Lo; L J Applegate; J N Carberry; L G Solti-Bohman; J W House; D E Brackmann; V Waluch; J C Li Journal: Radiology Date: 1993-10 Impact factor: 11.105
Authors: Daniel Choo; Lawrence Shotland; Maryann Mastroianni; Gladys Glenn; Carter van Waes; W Marston Linehan; Edward H Oldfield Journal: J Neurosurg Date: 2004-03 Impact factor: 5.115
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: H Jeffrey Kim; Marygrace Hagan; John A Butman; Martin Baggenstos; Carmen Brewer; Christopher Zalewski; W Marston Linehan; Russell R Lonser Journal: Laryngoscope Date: 2012-10-15 Impact factor: 3.325