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Unusual clinical and MRI features of a cerebellopontine angle medulloepithelioma. Case report and review of literature.

Syal R, Reddy S J, Kumar R, Tyagi I, Abrar AA, Krishnani N, Mishra AM, Gupta RK

Department of Neurosurgery, Neurootology Unit, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, India. drrajansyal@yahoo.co.in

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.

Published 11 August 2006 in Pediatr Neurosurg, 42(5): 299-303.
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