Primary Intraspinal Primitive Neuroectodermal Tumor

Abstract

Primary spinal primitive neuroectodermal tumor (PNET) is a rare condition, 18 cases of which have been reported in the literature. In general, this tumor is treated with surgery followed by radiotherapy and chemotherapy, but prognosis is still poor. An 18-year-old female patient with an intradural, extramedullary mass at L3-L5 levels is presented in this report. This is the first female patient with primary spinal PNET at lumbar region, second patient with spinal nerve root origin, and third one with intradural, extramedullary localization ever reported in the literature. After surgery, she was treated with craniospinal radiotherapy and four cycles of combination chemotherapy regimen consisting of vincristine, cyclophosphamide, doxorubicin alternated with ifosfamide, and VP-16. Currently, she is asymptomatic and alive at 25 months. The histopathologic, radiologic, and clinical findings of the patient are presented and relevant literature is reviewed.