Abstract
Summary: Although these entities are histologically similar, recent advances in molecular genetics have allowed the distinction of central nervous system extraosseous Ewing sarcoma (CNS-EES) from central primitive neuroectodermal tumors (c-PNET) including medulloblastoma and supratentorial PNET. We present 2 cases of pathologically confirmed CNS-EES. Knowledge of CNS-EES as a distinct entity enables the neuroradiologist to suggest the proper diagnosis and the need for special immuno-histochemical and molecular studies to confirm the diagnosis. Because treatment and prognosis are vastly different, the proper diagnosis of CNS-EES versus c-PNET is critical.
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