Primary spinal primitive neuroectodermal tumor: case series and review of the literature.

Abstract:

:Primary spinal primitive neuroectodermal tumor (PSPNET) is extremely rare and only 25 cases have been reported in the world literature so far. Three patients of 8, 9 and 18 years of age, who presented with variable grades of neurological deficit were diagnosed as having a dorsal intramedullary lesion, a holocord lesion and cervical extradural tumor with extraspinal extension, respectively, and were operated at our institute. The histopathology of all 3 children revealed PNET. The clinical course, image characteristics and outcome of the 3 children are described, and the relevant literature is reviewed. The following conclusions were drawn from the present study and review of the literature. PNET may manifest itself as a primary lesion of the spine unlike the more common drop metastases from an intracranial lesion. PSPNET may be intramedullary, intradural and extradural with variable extraspinal extension. PSPNET may present as holocord intramedullary lesion, an entity which has not been described earlier. These lesions have a short history, significant neurological deficits and rapid course of illness. PSPNET, though an established entity, did not find a place in the WHO 2000 classification of CNS tumors. Hence its status has to be defined.

journal_name

Pediatr Neurosurg

journal_title

Pediatric neurosurgery

authors

Kumar R,Reddy SJ,Wani AA,Pal L

doi

10.1159/000097517

subject

Has Abstract

pub_date

2007-01-01 00:00:00

pages

1-6

issue

1

eissn

1016-2291

issn

1423-0305

pii

000097517

journal_volume

43

pub_type

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