Intraparenchymal central nervous system Ewing’s sarcoma. A case treated according to the modified Euro-EWING protocol

Marie-Amelyne Le Rouzic, Julie Valduga, Ludovic Mansuy, Emmanuelle Schmitt, Olivier Klein, Valérie Bernier, Pascal Chastagner


Ewing's sarcoma (ES) is the second most common malignant bone tumor in children and young adults. ES may also occur as a primary soft tissue neoplasm without involvement of the bone and is then referred to as extraosseous ES (EES). The paediatric central nervous system extraosseous Ewing's sarcoma (CNS-EES) is an extremely rare entity since less than 20 cases have been reported in literature. Moreover, only less than 10 cases correspond to brain parenchyma primary tumors. These cases have been often misdiagnosed with other varieties of primary brain tumors, particularly those which were called PNET (Primitive Neuro-Ectodermal Tumor). We present a case of true intraparenchymal CNS-EES and a comparison of epidemiology, behaviour, treatment, and prognosis of CNS-EES and intracranial PNET cases.


extraosseous Ewing’s sarcoma; central nervous system; surgery; chemotherapy; radiotherapy; child

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Copyright (c) 2017 Marie-Amelyne Le Rouzic, Julie Valduga, Ludovic Mansuy, Emmanuelle Schmitt, Olivier Klein, Valérie Bernier, Pascal Chastagner

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ISSN: 2360-6975