Abstract: Abstract: Objective To investigate the clinical characteristics, diagnosis, pathological features and treatment of primary intraspinal peripheral primitive neuroectodermal tumor (pPNET). Methods The clinical materials of 2 intraspinal PNET，such as image data，neurosurgical treatment and histopathology features were analyzed retrospectively. Results Two PNETs were isointense on T1-weighted images and T2-weighted images, and with Gd-DTPA enhancement apparently. Both of them were treated by surgery and underwent subtotal resection. They were identified peripheral primitive neuroectodermal tumor by histopathological examination and immunohistochemical staining. Furthermore, they underwent radiotherapy and chemotherapy. Both of them were followed up. Conclusions Intraspinal PNET is very rare and frequently located at lumbar/lumbosacral spinal level and most of them are peripheral PNET. The patient has short duration of symptoms and poor prognosis. Treatment proposals are based on histopathological results. Newer therapeutic strategy will improve the survival of patient.

Key words: Key words: primitive neuroectodermal tumor, intraspinal