Zeitschrift für Wirbelsäulen- und Neurochirurgie

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

Ali Shirzadi, Debraj Mukherjee, Michael J. Alexander, Lee Tan, Serguei I. Bannykh, Rick A. Friedman, Keith L. Black and Moise Danielpour

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.