A series of 62 children with cerebral arteriovenous malformations admitted to our department in the course of 17 years (1975-1992) was reviewed in a retrospective study. In 54 cases hemorrhagic stroke was the first presenting symptom, followed by epilepsy in five cases. On admission 26 children presented with a neurological deficit, and 21 were admitted with a grade 3 status according to Botterell. Fifty-one malformations were supratentorial (41 hemispheric, 10 deep-seated) while 11 were infratentorial. According to Mori's criteria, 28 lesions were small, 19 medium, and 15 large. Fifty-two children were operated on, with total excision of the malformation achieved in 47 cases. In two children the malformation recurred. The evolution of neurological disorders has been studied with a mean follow-up of 8.5 years. Fifty patients had a satisfactory outcome on the Glasgow Outcome Scale. Four children died. These results were compared with those reported elsewhere in the pediatric literature.

Ewing’s sarcoma (ES) of the skull is rare. Herein, we present 2 cases of ES that involved the cranium in young children. In one case, the lesion originated in the petrous temporal bone; in the other, the frontal bone. Both children were acutely compromised neurologically by signs and symptoms of raised intracranial pressure. In both cases, radiographs revealed massive tumors affecting the skull. Neurosurgical resection of the tumor was undertaken in both instances, and the diagnosis of ES was confirmed by immunohistochemistry, cytogenetic analysis (translocation 11;22), spectral karyotyping and RT-PCR (demonstration of a EWS/FLI1 fusion transcript). Following aggressive surgical resection, both children received intensive chemotherapy. No child has received radiation therapy. One child is alive and well 8 years after diagnosis without any evidence of residual disease. The other is currently undergoing chemotherapy for her tumor. The principles involved in the management of children with cranial-based ES are discussed. These 2 cases serve to illustrate the fact that even children with massive ES tumors of the cranium may be salvaged with aggressive combination therapy.

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