European Archives of Oto-Rhino-Laryngology

, Volume 270, Issue 4, pp 1551–1557 | Cite as

Endolymphatic sac tumors: experience of three cases

  • Pierre-Louis Bastier
  • Erwan de Mones
  • Magali Marro
  • Wael Elkhatib
  • Valérie Franco-Vidal
  • Dominique Liguoro
  • Vincent Darrouzet
Case Report

Abstract

Objectives

To describe the clinical features, radiological findings, treatment and outcomes of three cases of endolymphatic sac tumors (ELST).

Methods

Retrospective analysis of three cases of ELST.

Results

The first patient had a large ELST invading the labyrinth after a long history of vertigo. He was recurrence-free 1 year after retrolabyrinthine surgical removal. In the second case, an acute peripheral facial nerve paralysis associated with ipsilateral sensorineural hearing loss led to the diagnosis. A translabyrinthine approach was used to remove the tumor, which recurred three times over 10 years. The third patient was a young woman suffering from von Hippel–Lindau (VHL) disease and referred for a sudden sensorineural hearing loss due to an intralabyrinthine hemorrhage secondary to a 2 mm-large endolymphatic sac-confined ELST. Her hearing was totally lost after the deafness recurred 1 month after this first episode. MRI demonstrated a small bilateral ELST. The patient refused surgery on the deaf side.

Conclusion

ELST are difficult to diagnose due to the wide variety of their presentations. Patients with ELST should be screened for VHL disease. Dural invasion and tumor hypervascularization increase the risk of local recurrences after surgery. Early surgical resection may lead to complete tumor removal and inner ear preservation.

Keywords

Endolymphatic sac tumor Von Hippel–Lindau disease Intralabyrinthine hemorrhage Endolymphatic hydrops 

References

  1. 1.
    Heffner DK (1989) Low-grade adenocarcinoma of probable endolymphatic sac origin: a clinicopathologic study of 20 cases. Cancer 64(11):2292–2302PubMedCrossRefGoogle Scholar
  2. 2.
    Yilmaz I, Bolat F, Demirhan B et al (2008) Endolymphatic sac papillary tumor: a case report and review. Auris Nasus Larynx 35(2):276–281PubMedCrossRefGoogle Scholar
  3. 3.
    Bambakidis NC, Megerian CA, Ratcheson RA (2004) Differential grading of endolymphatic sac tumor extension by virtue of von Hippel–Lindau disease status. Otol Neurotol 25(5):773–781PubMedCrossRefGoogle Scholar
  4. 4.
    Kempermann G, Neumann HP, Volk B (1998) Endolymphatic sac tumours. Histopathology 33(1):2–10PubMedCrossRefGoogle Scholar
  5. 5.
    Lonser RR, Baggenstos M, Kim HJ et al (2008) The vestibular aqueduct: site of origin of endolymphatic sac tumors. J Neurosurg 108(4):751–756PubMedCrossRefGoogle Scholar
  6. 6.
    Barnes EL, Evesin JW, Reichart P et al (2005) Pathology and genetics of head and neck tumours. In: Kleihues P, Sobin LH (eds) World Health Organisation classification of tumours. IARC Press, LyonGoogle Scholar
  7. 7.
    Manski TJ, Heffner DK, Glenn GM et al (1997) Endolymphatic sac tumors. A source of morbid hearing loss in von Hippel–Lindau disease. JAMA 277(18):1461–1466PubMedCrossRefGoogle Scholar
  8. 8.
    Lonser RR, Glenn GM, Walther M et al (2003) von Hippel–Lindau disease. Lancet 361(9374):2059–2067PubMedCrossRefGoogle Scholar
  9. 9.
    Diaz RC, Amjad EH, Sargent EW et al (2007) Tumors and pseudotumors of the endolymphatic sac. Skull Base 17(6):379–393PubMedCrossRefGoogle Scholar
  10. 10.
    Kim WY, Kaelin WG (2004) Role of VHL gene mutation in human cancer. J Clin Oncol 22(24):4991–5004PubMedCrossRefGoogle Scholar
  11. 11.
    Kawahara N, Kume H, Ueki K et al (1999) VHL gene inactivation in an endolymphatic sac tumor associated with von Hippel–Lindau disease. Neurology 53(1):208–210PubMedCrossRefGoogle Scholar
  12. 12.
    Peyre M, Gaillard S, van Effenterre R et al (2011) Conservative management of endolymphatic sac tumors in von Hippel–Lindau disease: case report. Acta Neurochir (Wien) 153(1):42–47 (discussion 47)CrossRefGoogle Scholar
  13. 13.
    Bell D, Gidley P, Levine N et al (2011) Endolymphatic sac tumor (aggressive papillary tumor of middle ear and temporal bone): sine qua non radiology–pathology and the University of Texas MD Anderson Cancer Center experience. Ann Diagn Pathol 15(2):117–123PubMedCrossRefGoogle Scholar
  14. 14.
    Codreanu C, Tran Ba Huy P (2010) Isolate vertigo crisis revealing an endolymphatic sac tumor. Rom J Morphol Embryol 51(2):387–389Google Scholar
  15. 15.
    Bambakidis NC, Rodrigue T, Megerian CA et al (2005) Endolymphatic sac tumor metastatic to the spine. Case report. J Neurosurg Spine 3(1):68–70PubMedCrossRefGoogle Scholar
  16. 16.
    Jagannathan J, Butman JA, Lonser RR et al (2007) Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. J Neurosurg 107(2):421–425PubMedCrossRefGoogle Scholar
  17. 17.
    Butman JA, Kim HJ, Baggenstos M et al (2007) Mechanisms of morbid hearing loss associated with tumors of the endolymphatic sac in von Hippel–Lindau disease. JAMA 298(1):41–48PubMedCrossRefGoogle Scholar
  18. 18.
    Kitahara T, Maekawa C, Kizawa K, Kamakura T, Horii A, Inohara H (2011) Endolymphatic sac tumor with overexpression of V2 receptor mRNA and inner ear hydrops. Acta Otolaryngol 131(9):951–957PubMedCrossRefGoogle Scholar
  19. 19.
    Megerian CA, Haynes DS, Poe DS, Choo DI, Keriakas TJ, Glasscock ME 3rd (2002) Hearing preservation surgery for small endolymphatic sac tumors in patients with von Hippel–Lindau syndrome. Otol Neurotol 23(3):378–387. (Review)Google Scholar
  20. 20.
    Muchnik C, Hildesheimer M, Rubinstein M, Arenberg IK (1989) Low frequency air–bone gap in Menière’s disease without middle ear pathology. A preliminary report. Am J Otol 10(1):1–4PubMedCrossRefGoogle Scholar
  21. 21.
    Safatle PPN, Farage L, Sampaio A et al (2009) Endolymphatic sac tumor and von Hippel–Lindau disease in a single family. Arq Neuropsiquiatr 67(4):1097–1099PubMedCrossRefGoogle Scholar
  22. 22.
    Maher ER, Neumann HP, Richard S (2011) von Hippel–Lindau disease: a clinical and scientific review. Eur J of Hum Genet 19(6):617–623CrossRefGoogle Scholar
  23. 23.
    Jagannathan J, Lonser RR, Stanger RA et al (2007) Cochlear implantation for hearing loss associated with bilateral endolymphatic sac tumors in von Hippel–Lindau disease. Otol Neurotol 28(7):927–930PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Pierre-Louis Bastier
    • 1
    • 3
  • Erwan de Mones
    • 1
  • Magali Marro
    • 1
  • Wael Elkhatib
    • 1
  • Valérie Franco-Vidal
    • 1
  • Dominique Liguoro
    • 2
  • Vincent Darrouzet
    • 1
  1. 1.Otolaryngology and Skull Base Surgery DepartmentUniversity Bordeaux SegalenBordeauxFrance
  2. 2.Department of NeurosurgeryUniversity Bordeaux SegalenBordeauxFrance
  3. 3.Service ORLHôpital PellegrinBordeaux CedexFrance

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