Primary intraspinal primitive neuroectodermal tumor: case report of a tumor arising from the sacral spinal nerve root and review of the literature.
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.
Yavuz
A Aydin
AA
Department of Radiation Oncology, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey.
Yaris
Nilgun
N
Yavuz
Melek N
MN
Sari
Ahmet
A
Reis
A Kadir
AK
Aydin
Fazil
F
D000293:Adolescent; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008159:Lumbar Vertebrae; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D013120:Spinal Cord Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D013126:Spinal Nerve Roots