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Case Reports in Neurological Medicine
Volume 2012 (2012), Article ID 396097, 4 pages
http://dx.doi.org/10.1155/2012/396097
Case Report

A Tragical Paediatric Case History of Intraorbital and Intracranial Epithelioid Hemangioendothelioma

Department of Neurosurgery, Mohammed VI University Medical Centre, and Cadi Ayyad University, Marrakesh, Morocco

Received 22 September 2012; Accepted 10 October 2012

Academic Editors: J. E. Cohen and I. L. Simone

Copyright © 2012 K. Aniba et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Epithelioid hemangioendothelioma (EHE) is a rare tumor of intermediate malignancy. We report a case of intracranial and intraorbitar EHE. A 3-year-old girl presented with a 3-month history of progressive left exophthalmia. Neuroradiologic imaging (CT scan and MRI) showed an intraorbitar process with an intense enhancement extending to temporal fossa, ethmoidal bone, nasal fossa, maxillary sinus, and cavernous sinus. The angiogram was normal. The tumor was operated through subfrontal approach but only a partial resection was performed. The histological diagnosis was epithelioid hemangioendothelioma. The patient was neurologically intact 2 months after surgery without exophtalmia. However 4 months after surgery he displayed a fall of the right eye vision with intense headache. Control CT scan showed persistence of important tumoral residue. Epithelioid hemangioendothelioma is a hemorrhagic tumor. Total removal must be possible. Otherwise, we recommend a complementary chemoradiotherapy and close followup. We propose this interesting case history of a tragical evolution of EHE in contradiction with what has already been reported.