Primary intramedullary primitive neuroectodermal tumor of the spinal cord: case report and review of the literature

Primary intraspinal primitive neuroectodermal tumors (PNETs) are rare. We report a case and review the literature. A 22-year-old woman presented with rapidly progressive paraparesis and neurogenic bladder. Preoperative computed tomography myelograms revealed a complete block at T12-L1, consistent with an intramedullary lesion. An urgent operation was performed with gross total tumor removal. The pathological findings were consistent with a PNET. Recurrence was noted within 10 weeks of surgery and has been somewhat responsive to chemotherapy and radiotherapy thus far. A review of the English literature shows that only 13 cases of primary intraspinal PNETs have been reported to date, and the present case is the second one in which the tumor was purely intramedullary. Most of the reported patients survived less than 2 years. Primary intraspinal PNETs are rare tumors and carry a poor prognosis.