Intraspinal primitive neuroectodermal tumour: Report of two cases and review of the literature

Two patients with primary intraspinal primitive neuroectodermal tumour are presented. In a 32-year-old man, the tumour evolved intradurally from a sac ral nerve root. Despite repeated surgery and radiochemotherapy, the patient suffered multiple intraspinal tumour relapses and intracranial seedings, a nd died 29 months after the first diagnosis. In a 17-year-old male adolesce nt, the tumour was located in the lumbar epidural space, extending into the paraspinal muscles. Following resection and radiochemotherapy, the patient is free from disease 23 months after the initial presentation. The clinica l, radiological, histopathological and cytogenetic findings of both patient s are presented and the relevant literature is reviewed. Particular attenti on is given to the histogenetic relationship between peripheral primitive n euroectodermal tumour and Ewing's sarcoma.