Journal of Spine & NeurosurgeryISSN: 2325-9701

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Case Report, J Spine Neurosurg Vol: 2 Issue: 1

An Apparent Sporadic Endolymphatic Sac Tumor in a 14-Year-Old Boy

Ali Shirzadi1, Debraj Mukherjee1*, Michael J. Alexander1, Lee Tan2, Serguei I. Bannykh1, Rick A. Friedman3, Keith L. Black1 and Moise Danielpour1
1Maxine Dunitz Neurosurgical Institute, Department of Neurosurgery, Cedars- Sinai Medical Center 8631 W. 3rd Street; Suite 800 East, Los Angeles, CA 90048, USA
2 Department of Neurosurgery, Rush University Medical Centre, 1653 W. Congress Parkway Chicago, Illinois 60612, USA
3House Ear Institute, 2100 W. 3rd Street, Suite 111, Los Angeles, CA 90057, USA
Corresponding author : Debraj Mukherjee
Department of Neurosurgery, Maxine Dunitz Neurosurgical Institute, Cedars-Sinai Medical Center, 8631 W. 3rd Street, Suite 800 East, Los Angeles, CA 90048 USA, Tel: 310-729-1247; Fax: 310-423-0810
Received: October 16, 2012 Accepted: December 14, 2012 Published: December 17, 2012
Citation: Shirzadi A, Mukherjee D, Alexander MJ, Tan L, Bannykh SI, et al. (2013) An Apparent Sporadic Endolymphatic Sac Tumor in a 14-Year-Old Boy. J Spine Neurosurg 2:1. doi:10.4172/2325-9701.1000105


An Apparent Sporadic Endolymphatic Sac Tumor in a 14-Year-Old Boy

Endolymphatic sac tumors (ELSTs) are rare, low-grade, histologically benign tumors arising from the epithelial lining of the endolymphatic duct or sac. They are slow growing but locally invasive and can be associated with von Hippel-Lindau disease. While there are cases in the medical literature documenting this type of tumor in adults, reports of ELSTs in the pediatric population are limited.The authors report the fifth reported case of a pediatric ELST in the English literature. A 14-year-old Jehovah’s Witness presented with a progressive two-year history of right-sided hearing loss, balance problems, gait difficulty, and bleeding behind the right tympanic membrane. Computed topography (CT) and magnetic resonance imaging (MRI) of the brain and temporal bone revealed a large lesion eroding into the petrous and mastoid bones with extension abutting the brain stem and invading the right transverse sinus.

Keywords: Endolymphatic sac tumor; Temporal/Petrous/Mastoid bone tumor; Von Hippel-Lindau

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