Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage

Case report

View More View Less
Restricted access

Purchase Now

USD  $45.00

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $505.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00
Print or Print + Online

✓ Endolymphatic sac tumors (ELSTs) are locally invasive neoplasms that arise in the posterior petrous bone and are associated with von Hippel–Lindau (VHL) disease. These tumors cause symptoms even when microscopic in size (below the threshold for detectability on imaging studies) and can lead to symptoms such as hearing loss, tinnitus, vertigo, and facial nerve dysfunction. While the mechanisms of audiovestibular dysfunction in patients harboring ELSTs are incompletely understood, they have critical implications for management. The authors present the case of a 33-year-old man with VHL disease and a 10-year history of progressive tinnitus, vertigo, and left-sided hearing loss. Serial T1-weighted magnetic resonance (MR) imaging and computed tomography scans revealed no evidence of tumor, but fluid attenuated inversion recovery (FLAIR) MR imaging sequences obtained after hearing loss demonstrated evidence of left intralabyrinthine hemorrhage. On the basis of progressive disabling audiovestibular dysfunction (tinnitus and vertigo), FLAIR imaging findings, and VHL disease status, the patient underwent surgical exploration of the posterior petrous region, and a small (2-mm) ELST was identified and completely resected. Postoperatively, the patient had improvement of the tinnitus and vertigo. Intralabyrinthine hemorrhage may be an early and the only neuroimaging sign of an ELST in patients with VHL disease and audiovestibular dysfunction. These findings support tumor-associated hemorrhage as a mechanism underlying the audiovestibular dysfunction associated with ELSTs.

Abbreviations used in this paper:CT = computed tomography; ELST = endolymphatic sac tumor; FLAIR = fluid attenuated inversion recovery; MR = magnetic resonance; VHL = von Hippel–Lindau.

JNS + Pediatrics - 1 year subscription bundle (Individuals Only)

USD  $505.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $600.00

Contributor Notes

Address reprint requests 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.
  • 1

    Choo D, , Shotland L, , Mastroianni M, , Glenn G, , van Waes C, & Linehan WM, : Endolymphatic sac tumors in von Hippel-Lindau disease. J Neurosurg 100:480487, 2004

    • Search Google Scholar
    • Export Citation
  • 2

    Gaeta M, , Blandino A, , Minutoli F, & Pandolfo I: Sudden unilateral deafness with endolymphatic sac adenocarcinoma: MRI. Neuroradiology 41:799801, 1999

    • Search Google Scholar
    • Export Citation
  • 3

    Heffner DK: Low-grade adenocarcinoma of probable endolymphatic sac origin A clinicopathologic study of 20 cases. Cancer 64:22922302, 1989

    • Search Google Scholar
    • Export Citation
  • 4

    Horiguchi H, , Sano T, , Toi H, , Kageji T, , Hirokawa M, & Nagahiro S: Endolymphatic sac tumor associated with a von Hippel-Lindau disease patient: an immunohistochemical study. Mod Pathol 14:727732, 2001

    • Search Google Scholar
    • Export Citation
  • 5

    Khan KM, , Sarfaraz N, , Siddiqui S, & Nawaz H: Immunohistochemical localization of G protein betagamma subunits in the lateral wall of the rat cochlea. J Anat 208:205218, 2006

    • Search Google Scholar
    • Export Citation
  • 6

    Kilickesmez O: Endolymphatic sac tumor in a patient with von Hippel-Lindau disease: MR imaging findings. Diagn Interv Radiol 12:1416, 2006

    • Search Google Scholar
    • Export Citation
  • 7

    Kim HJ, , Butman JA, , Brewer C, , Zalewski C, , Vortmeyer AO, & Glenn G, : Tumors of the endolymphatic sac in patients with von Hippel-Lindau disease: implications for their natural history, diagnosis, and treatment. J Neurosurg 102:503512, 2005

    • Search Google Scholar
    • Export Citation
  • 8

    Latif F, , Tory K, , Gnarra J, , Yao M, , Duh FM, & Orcutt ML, : Identification of the von Hippel-Lindau disease tumor suppressor gene. Science 260:13171320, 1993

    • Search Google Scholar
    • Export Citation
  • 9

    Lonser RR, , Glenn GM, , Walther M, , Chew EY, , Libutti SK, & Linehan WM, : von Hippel-Lindau disease. Lancet 361:20592067, 2003

  • 10

    Lonser RR, , Kim HJ, , Butman JA, , Vortmeyer AO, , Choo DI, & Old-field EH: Tumors of the endolymphatic sac in von Hippel-Lindau disease. N Engl J Med 350:24812486, 2004

    • Search Google Scholar
    • Export Citation
  • 11

    Lonser RR, , Weil RJ, , Wanebo JE, , DeVroom HL, & Oldfield EH: Surgical management of spinal cord hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 98:106116, 2003

    • Search Google Scholar
    • Export Citation
  • 12

    Manski TJ, , Heffner DK, , Glenn GM, , Patronas NJ, , Pikus AT, & Katz D, : Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel-Lindau disease. JAMA 277:14611466, 1997

    • Search Google Scholar
    • Export Citation
  • 13

    Megerian CA, , McKenna MJ, , Nuss RC, , Maniglia AJ, , Ojemann RG, & Pilch BZ, : Endolymphatic sac tumors: histopathologic confirmation, clinical characterization, and implication in von Hippel-Lindau disease. Laryngoscope 105:801808, 1995

    • Search Google Scholar
    • Export Citation
  • 14

    Mukherji SK, , Albernaz VS, , Lo WW, , Gaffey MJ, , Megerian CA, & Feghali JG, : Papillary endolymphatic sac tumors: CT, MR imaging, and angiographic findings in 20 patients. Radiology 202:801808, 1997

    • Search Google Scholar
    • Export Citation
  • 15

    Palmer JM, , Coker NJ, & Harper RL: Papillary adenoma of the temporal bone in von Hippel-Lindau disease. Otolaryngol Head Neck Surg 100:6468, 1989

    • Search Google Scholar
    • Export Citation
  • 16

    Poe DS, , Tarlov EC, , Thomas CB, & Kveton JF: Aggressive papillary tumors of temporal bone. Otolaryngol Head Neck Surg 108:8086, 1993

  • 17

    Rakheja D, , Kapur P, , Milchgrub S, & Hoang MP: Pathologic quiz case: a woman with right facial nerve paralysis. Endolymphatic sac tumor associated with von Hippel-Lindau disease. Arch Pathol Lab Med 127:13871388, 2003

    • Search Google Scholar
    • Export Citation
  • 18

    Rizvi SS, & Gibbin KP: Effect of transverse temporal bone fracture on the fluid compartment of the inner ear. Ann Otol Rhinol Laryngol 88:741748, 1979

    • Search Google Scholar
    • Export Citation
  • 19

    Sando I, & Egami T: Inner ear hemorrhage and endolymphatic hydrops in a leukemic patient with sudden hearing loss. Ann Otol Rhinol Laryngol 86:518524, 1977

    • Search Google Scholar
    • Export Citation
  • 20

    Shibata T, , Hibino H, , Doi K, , Suzuki T, , Hisa Y, & Kurachi Y: Gastric type H+, K+-ATPase in the cochlear lateral wall is critically involved in formation of the endocochlear potential. Am J Physiol Cell Physiol 291:C1038C1048, 2006

    • Search Google Scholar
    • Export Citation
  • 21

    Sugiura M, , Naganawa S, , Teranishi M, & Nakashima T: Three-dimensional fluid-attenuated inversion recovery magnetic resonance imaging findings in patients with sudden sensorineural hearing loss. Laryngoscope 116:14511454, 2006

    • Search Google Scholar
    • Export Citation
  • 22

    Wanebo JE, , Lonser RR, , Glenn GM, & Oldfield EH: The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 98:8294, 2003

    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 324 136 8
Full Text Views 163 11 3
PDF Downloads 73 7 2
EPUB Downloads 0 0 0