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Optimum treatment for primary intracranial Ewing sarcoma
Published in Baylor University Medical Center Proceedings, 2020
Sasha G. Howell, Kristopher A. Lyon, David Garrett, Jason H. Huang, Ekokobe Fonkem
Ewing sarcoma (ES) is an aggressive neuroectodermal neoplasm that frequently manifests in children and young adults. ES tumors without osseous involvement are currently classified as extraosseous ES and were previously designated as primitive neuroectodermal tumors, World Health Organization grade IV.1,2 Histopathological identification of extraosseous ES tumors relies on the presence of a balanced translocation involving chromosomes 11 and 22 or 21 and 22. Extraosseous ES cells characteristically display dense expression of gene product MIC-2 membrane protein (CD99).3 Approaches to the chemotherapeutic treatment, radiation, and overall prognosis are thought to differ greatly between extraosseous ES and other embryonal tumors.4 Here we present a case of adult extraosseous ES arising from the pineal region with third and fourth ventricular invasion and evidence of leptomeningeal spread.