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These tumors are in the category of ES family of tumors, together with ES of bone, extraosseous ES, and primitive neuroectodermal tumor.

The most frequently involved sites are the craniospinal vault and cauda equina, while the peripheral nerve location is extremely rare only 11 cases mentioned in the literature to date. A review of the literature about diagnostic and treatment management is presented.

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For this reason, a multidisciplinary setting with a team of neurosurgeons, orthopedic specialists, radiologists, pathologists, and oncologists should manage these cases as soon as a pathologic diagnosis is available.

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