Ewing’s Sarcoma of the Cranial Vault Revealed by Macrocrania

Abstract

Ewing’s sarcomas are a heterogeneous group of aggressive tumors affecting mostly children and young adults. They account for 10% of primary malignancies of the bones and 3% of all malignancies in children. They mainly affect the long bones, pelvis, and ribs. Cranial vault localization is extremely rare and indeed account for less than 1% of all localizations in the body. We are reporting the unusual case of a 3-year-old child who presented to our unit with a macrocrania which had been evolving for 2 months prior to his admission, associated with sporadic vomiting and a right frontal swelling that was gradually installed. The cerebral computed tomography scan showed a right frontoparietal lytic tissular process, heterogeneously enhanced after injection of contrast material; with dural invasion and intraparenchymal extension. The child was biopsied and the pathological examination concluded Ewing’s sarcoma. The evolution was fatal with the passing of the child 2 weeks after the biopsy.

Keywords

• ewing’s sarcoma
• cranial vault
• macrocrania