Endolymphatic sac tumors

Cameron C Wick; Nauman F Manzoor; Maroun T Semaan; Cliff A Megerian

doi:10.1016/j.otc.2014.12.006

Endolymphatic sac tumors

Otolaryngol Clin North Am. 2015 Apr;48(2):317-30. doi: 10.1016/j.otc.2014.12.006. Epub 2015 Jan 31.

Authors

Cameron C Wick¹, Nauman F Manzoor¹, Maroun T Semaan², Cliff A Megerian³

Affiliations

¹ Ear, Nose, and Throat Institute, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH, USA.
² Ear, Nose, and Throat Institute, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH, USA; Otology, Neurotology, and Balance Disorders, Department of Otolaryngology - Head and Neck Surgery, Ear, Nose, and Throat Institute, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH, USA.
³ Ear, Nose, and Throat Institute, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH, USA; Otology, Neurotology, and Balance Disorders, Department of Otolaryngology - Head and Neck Surgery, Ear, Nose, and Throat Institute, University Hospitals Case Medical Center, Case Western Reserve University School of Medicine, Cleveland, OH, USA. Electronic address: Cliff.Megerian@UHhospitals.org.

PMID: 25650230
DOI: 10.1016/j.otc.2014.12.006

Abstract

Endolymphatic sac tumors (ELST) are slow-growing, locally aggressive, low-grade malignancies that originate from the epithelium of the endolymphatic duct and sac. ELST often present with sensorineural hearing loss, tinnitus, and vertigo, which may mimic Meniere disease. Large tumors may present with additional cranial neuropathies. Management is primarily via microsurgical excision. Radiation therapy has a limited role for residual or unresectable disease. Early detection may enable hearing preservation techniques. ELST have an association with von Hippel-Lindau disease.

Keywords: Endolymphatic sac tumors; Hearing preservation; Meniere syndrome; Von Hippel–Lindau.

Publication types

Review

MeSH terms

Endolymphatic Sac / pathology
Endolymphatic Sac / surgery*
Hearing Loss, Sensorineural
Humans
Magnetic Resonance Imaging
Meniere Disease / diagnosis
Neoplasm Staging
Neoplasms / complications
Neoplasms / pathology*
Neoplasms / surgery*
Tinnitus
Tomography, X-Ray Computed
Vertigo
von Hippel-Lindau Disease / complications*