title: Primary Ewing's sarcoma of the skull in children. Utility of molecular diagnostics, surgery and adjuvant therapies.
abstract: 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.
authors.affiliation: Division of Neurosurgery, The Hospital for Sick Children, The University of Toronto, Canada.
authors.forename: C G
authors.initials: CG
authors.lastname: Carlotti
authors.forename: J M
authors.initials: JM
authors.lastname: Drake
authors.forename: J P
authors.initials: JP
authors.lastname: Hladky
authors.forename: I
authors.initials: I
authors.lastname: Teshima
authors.forename: L E
authors.initials: LE
authors.lastname: Becker
authors.forename: J T
authors.initials: JT
authors.lastname: Rutka
mesh_terms: D000208:Acute Disease; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D002533:Cerebral Angiography; D005260:Female; D006801:Humans; D007223:Infant; D007621:Karyotyping; D008279:Magnetic Resonance Imaging; D008297:Male; D020133:Reverse Transcriptase Polymerase Chain Reaction / Q000379:methods; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D012888:Skull Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D014057:Tomography, X-Ray Computed
chemical_list: D000970:Antineoplastic Agents