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Adv Ther. 2018 Jun 19. doi: 10.1007/s12325-018-0730-0. [Epub ahead of print]

Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone.

Author information

1
Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL, USA. mendwm@shands.ufl.edu.
2
Instituto de Investigacion Sanitaria del Principado de Asturias and CIBERONC, ISCIII, Oviedo, Spain.
3
Instituto Universitario de Oncología del Principado de Asturias, University of Oviedo, Oviedo, Spain.
4
Department of Pathology, Faculty of Medicine in Plzen, Charles University in Prague, Plzeň, Czech Republic.
5
Department of Radiation Oncology, Institute of Oncology, Ljubljana, Slovenia.
6
Department of Pathology, Liverpool Clinical Laboratories, University of Liverpool, Liverpool, UK.
7
Department of Pathology, Allegiance Health, Jackson, MI, USA.
8
Department of Pathology, MD Anderson Cancer Center, University of Texas, Houston, TX, USA.
9
University of Udine School of Medicine, Udine, Italy.
10
Coordinator of the International Head and Neck Scientific Group, Padua, Italy.

Abstract

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

PMID:
29923043
DOI:
10.1007/s12325-018-0730-0

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