The vestibular aqueduct: site of origin of endolymphatic sac tumors

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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 (≤ 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 (≤ 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.

Abbreviations used in this paper: CT = computed tomography; ELST = endolymphatic sac tumor; MR = magnetic resonance; RLPP = retrolabyrinthine posterior petrosectomy; VHL = von Hippel–Lindau.

Article Information

Address correspondence to: Russell R. Lonser, M.D., Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10, Room 5D37, Bethesda, Maryland 20892-1414. email: lonserr@ninds.nih.gov.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Characteristic imaging and histological findings from a 40-year-old patient with VHL disease who had a right-sided ELST and preoperative symptomatology that included right-sided hearing loss, tinnitus, and vertigo. A: Axial, T1-weighted, enhanced MR image demonstrating a small (3-mm) enhancing lesion (arrowhead) within the right vestibular aqueduct (external aperture of vestibular aqueduct, arrow). B: Corresponding axial unenhanced CT image demonstrating osseous erosion by tumor (arrowhead) within the proximal vestibular aqueduct (external aperture of vestibular aqueduct, arrowhead). Consistent with imaging findings, a small ELST was identified within the vestibular aqueduct. C: Hematoxylin and eosin staining demonstrates a papillary-cystic ELST. Original magnification × 20. D: Immunohistochemical staining for CD34 antigen (dark staining) demonstrates the intense vascularization characteristic of ELSTs. Original magnification × 20.

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    Serial imaging and histological findings in a 38-year-old patient with VHL disease that demonstrate the development of a left-sided ELST within the vestibular aqueduct. The patient presented in 2000 with acute onset of left-sided tinnitus. A: Axial, T1-weighted, enhanced MR imaging did not demonstrate evidence of an ELST. In 2002, the patient presented with worsening tinnitus and acute left-sided hearing loss. B: Axial, T1-weighted, enhanced MR imaging at that time demonstrated an enhancing tumor (arrow) within the proximal vestibular aqueduct. C: Corresponding axial, unenhanced CT imaging demonstrated tumor-associated erosion in the vestibular aqueduct (arrowhead). Consistent with imaging findings, a small ELST was identified within the vestibular aqueduct. D: Hematoxylin and eosin staining demonstrates a papillary-cystic ELST. Original magnification × 20.

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    Schematic illustration detailing the anatomy of the endolymphatic system and its relationship to surrounding petrous bone structure. The endolymphatic sac and duct are part of the membranous labyrinth of the inner ear. The endo-lymphatic duct is connected to the membranous labyrinth of the inner ear by the saccular and utricular ducts. The saccular and utricular ducts form the sinus of the endolymphatic duct. The sinus of the endolymphatic duct tapers and becomes the isthmus of the endolymphatic duct as it enters the bony vestibular aqueduct. The isthmus of the endolymphatic duct connects to the intraosseous portion (within the vestibular aqueduct) of the endolymphatic sac. Endolymphatic sac tumors arise from the endolymphatic epithelium of the endolymphatic duct and sac with the vestibular aqueduct (osseous portion, striped area). Distally, the extraosseous portion of the endolymphatic sac begins as the sac exits the aperture of the vestibular aqueduct. The extraosseous portion of the sac resides between the leaves of the posterior fossa dura mater on the posterior wall of the petrous ridge.

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