A Tragical Paediatric Case History of Intraorbital and Intracranial Epithelioid Hemangioendothelioma

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.