Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone

Adv Ther. 2018 Jul;35(7):887-898. doi: 10.1007/s12325-018-0730-0. Epub 2018 Jun 19.


An endolymphatic sac tumor (ELST) is a rare, indolent but locally aggressive tumor arising in the posterior petrous ridge. Patients present with sensorineural hearing loss and tinnitus. As the tumor progresses, patients may experience vertigo, ataxia, facial nerve paresis, pain and otorrhea. Most patients present in their 4th or 5th decade with a wide age range. Patients with von Hippel-Lindau disease have an increased likelihood of developing ELST. Histologically, ELST is a low-grade adenocarcinoma. As it progresses, it destroys bone and extends into adjacent tissues. The likelihood of regional or distant metastases is remote. The optimal treatment is resection with negative margins. Patients with positive margins, gross residual disease, or unresectable tumor are treated with radiotherapy or radiosurgery. Late recurrences are common, so long follow-up is necessary to assess efficacy. The likelihood of cure depends on tumor extent and is probably in the range of 50-75%.

Keywords: Endolymphatic sac tumor; Heffner tumor; Radiation therapy; Surgery; Temporal bone; von Hippel–Lindau disease.

Publication types

  • Review

MeSH terms

  • Adenocarcinoma* / pathology
  • Adenocarcinoma* / therapy
  • Disease Management
  • Disease Progression
  • Ear Neoplasms* / pathology
  • Ear Neoplasms* / therapy
  • Endolymphatic Sac / pathology*
  • Humans
  • Risk Factors
  • Temporal Bone / pathology*
  • von Hippel-Lindau Disease* / diagnosis
  • von Hippel-Lindau Disease* / epidemiology