Abstract
Ewing sarcomas (ES) and peripheral primitive neuroectodermal tumors (pPNET) are now thought to belong to the same tumor family. Ewing sarcoma family tumor (ESFT) members commonly originate in bones and soft tissues. However, a few published articles describe ESFT arising from cranial cavities. Pathologically, ES/pPNET are composed of small round cells. Unambiguous distinction between pPNET and other small round cell tumors, in particular central PNET, is of clinical significance. Definitive diagnoses of pPNET can be obtained through CD99 (MIC2 gene product) membrane positivities and molecular identifications of chromosomal rearrangements between EWS and ETS family genes. Multimodal approaches comprising surgical resections, radiotherapies, and chemotherapies are required for the treatment of ESFT. Decompressive medical measures are preferentially performed when epidural masses are compressing spinal cords. In cases of ES-induced brain herniations, emergent radiotherapies may serve as effective tools. We report a case of multiple disseminated intracranial ES/pPNET for which synthetic treatments were used.