Primary Ewing's sarcoma of the skull in children - Utility of molecular diagnostics, surgery and adjuvant therapies

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 origi nated in the petrous temporal bone; in the other, the frontal bone. Both ch ildren were acutely compromised neurologically by signs and symptoms of rai sed intracranial pressure. In both cases, radiographs revealed massive tumo rs affecting the skull. Neurosurgical resection of the tumor was undertaken in both instances, and the diagnosis of ES was confirmed by immunohistoche mistry, cytogenetic analysis (translocation 11;22), spectral karyotyping an d RT-PCR (demonstration of a EWS/FLI1 fusion transcript). Following aggress ive surgical resection, both children received intensive chemotherapy. No c hild has received radiation therapy. One child is alive and well 8 years af ter diagnosis without any evidence of residual disease. The other is curren tly undergoing chemotherapy for her tumor. The principles involved in the m anagement of children with cranial-based ES are discussed. These 2 cases se rve to illustrate the fact that even children with massive ES tumors of the cranium may be salvaged with aggressive combination therapy. Copyright (C) 2000 S. Karger AG, Basel.