Case Report

Unusual Clinical and MRI Features of a Cerebellopontine Angle Medulloepithelioma
Case Report and Review of Literature
Rajan Syal^{a, b}, Jaypal Reddy S^b, Raj Kumar^b, Isha Tyagi^{a, b}, A.A. Wani Abrar^b, Narender Krishnani^c, Asht M. Mishra^d, Rakesh K. Gupta<sup>d

a</sup>Neurootology Unit,
^bDepartment of Neurosurgery,
^cDepartment of Pathology, and
^dMR Section, Department of Radiology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, India

Address of Corresponding Author

Pediatric Neurosurgery 2006;42:299-303 (DOI: 10.1159/000094066)

  Key Words

• Primitive neuroectodermal tumor
• Cerebellopontine angle
• Medulloepithelioma
• Diffusion-weighted imaging
• Posterior fossa

  Abstract

We describe for the first time an unusual location and clinical presentation of medulloepithelioma, a rare embryonal tumor. A 5-year-old child presented with sudden onset of bilateral hearing loss. On imaging, the lesion appeared to be extra axial and was located in the right cerebello-pontine (CP) angle, extending into middle fossa along the trigeminal ganglion and in front of the brain stem into the opposite CP angle. It did not show any enhancement following contrast administration and had restricted diffusion on diffusion-weighted imaging, simulating an epidermoid. However, in vivo localized proton MR spectroscopy revealed a creatine peak dominated by a large choline resonance, peak of glycine with lactate/lipid and invisible N-acetylaspartate suggestive of a neoplastic lesion and not an epidermoid. Only subtotal resection could be performed and the patient had a stormy post-operative course due to extensive dissemination of the disease.

Copyright © 2006 S. Karger AG, Basel

  Author Contacts

Dr. Rajan Syal
Department of Neurosurgery, Neurootology Unit
Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Raibareily Road
Lucknow (UP) - 226 014 (India), Tel. +91 94 1547 1423, Fax +91 52 2277 8718
E-Mail drrajansyal@yahoo.co.in or rajkumar@sgpgi.ac.in

  Article Information

All authors contributed equally to the patient's management and the conception of this paper.

Received: May 20, 2005
Accepted after revision: October 17, 2005
Number of Print Pages : 5
Number of Figures : 3, Number of Tables : 0, Number of References : 15