PRIMARY INTRASPINAL PRIMITIVE NEUROECTODERMAL TUMORS: REPORT OF A CASE

Background – Primitive neuroectodermal tumors (PNETs) are rare neoplasms that are usually seen in children and frequently metastatize in the central nervous system. We present a case of primary intraspinal PNET. Methods – A 22-year-old woman presented with back pain and leg weakness. Magnetic resonance imaging of the spine revealed an intradural extramedullary lesion at the level of thoracic vertebra 12. Total tumor removal was performed and pathology was consistent with a PNET. Results – Within 4 weeks, there was a recurrence of symptoms with paraparesis at the original site and at the level of C1, T6, and T9. The PNET responded dramatically to radiotherapy. Conclusion – To date, only 14 cases of primary intraspinal PNET have been reported. The presented case is the second reported, in which the tumor was intradural and extramedullary. Intraspinal PNETs have a poor prognosis with reported cases surviving less than 2 years.