Sacral Primitive Neuroectodermal Tumor: case report and review of the literature

Pr imary intraspinal pr imit ive neuroecto dermal tumors (PNETs) are rare. We report a case of sacral PNET with review the literature. A 15 -yearold boy presented w ith progres sive lumbar area pain, lower leg numbness and neurogenic bladder. Preoperat ive magnet ic resonance imag ing revealed a sacral tumor with epidural, presacral, and sacral bone extensions. An urgent operation was performed with gross tumor removal. The pathological f indings were consistent with a PNET. This tumor is somewhat responsive to chemotherapy and radiotherapy thus far. A review of the English literatures showed that only some cases of primary intraspinal PNETs have been reported to date, and the present case is one of very rare cases reported in English literatures in which the tumors were of purely sacral origin sites. Most of the reported PNET patients survived less than 2 years. Primary intraspinal sacral PNETs are rare tumors and carry a poor prognosis. Primitive neuroectodermal tumors (PNETs) are malignant small cell neoplasms of children but can occur at any age. They most commonly occur in the cerebellum (medulloblastomas) but can also arise in the pineal gland, cerebrum, spinal cord, brain stem, and peripheral nerves. To date, only several cases of primary intraspinal PNETs have been reported in the English literature. Of these cases, only one is purely intramedullary; the remainder have been extradural, intradural extramedullary, or have arisen from the cauda equina. We report a case of PNET at the sacral area and review the published literatures of sacral PNETs.