Primary spinal intramedullary primitive neuroectodermal tumor

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Abstract

Though primitive neuroectodermal tumor has been considered to arise from a neoplastic transformation of primitive neuroepithelial cells with propensity to involve any part of the central nervous system, a primary intramedullary spinal primitive neuroectodermal tumor is very unusual. The authors describe a case of an 18-year-old female with conus intramedullary tumor diagnosed to be primary spinal primitive neuroectodermal tumor following histopathological examination after surgery. The diagnosis of such a tumor is very crucial as the management strategies for these are relatively unclear and are associated with a poorer outcome compared to the other common intramedullary spinal tumors.

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Most cited references 19

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

Raj Kumar Rauniyar, Priscilla Reddy, L Pal … (2006)

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. Copyright (c) 2007 S. Karger AG, Basel.

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The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors.

L Rorke (1982)

A simple classification system for central nervous system neoplasms occurring primarily in infancy and childhood and largely composed of undifferentiated neuroepithelial cells is proposed. Classification is based upon appearance of the tumor as determined by light microscopy, immunocytochemical techniques, and ultrastructural features without consideration for site of origin. This classification is based on the concept that neoplastic transformation of primitive neuroepithelial cells in subependymal zones at all levels of the central nervous system or pineal body may develop into tumors largely composed of similar cells. It therefore seems appropriate to call these neoplasms primitive neuroectodermal tumors and to use descriptive terms to indicate the direction of cellular differentiation, when it has occurred. Proposed terminology for the five subtypes of undifferentiated neuroepithelial round cell tumors is as follows: 1) Primitive neuroectodermal tumor, not otherwise specified (PNET, NOS), 2) PNET with glial differentiation, 3) PNET with ependymal differentiation, 4) PNET with neuronal differentiation, and 5) PNET with multi- or bipotential differentiation. If the tumor is located in the cerebellum, medulloblastoma may be added in parentheses; if in the pineal body, pineal parenchymal neoplasm may be added.

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

Boubacar S D Deme, L. Ang, G Skaf … (1997)

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.

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Author and article information

Journal

Journal ID (nlm-ta): J Pediatr Neurosci

Journal ID (iso-abbrev): J Pediatr Neurosci

Journal ID (publisher-id): JPN

Title: Journal of Pediatric Neurosciences

Publisher: Medknow Publications & Media Pvt Ltd (India )

ISSN (Print): 1817-1745

ISSN (Electronic): 1998-3948

Publication date (Print): Jan-Apr 2012

Volume: 7

Issue: 1

Pages: 67-69

Affiliations

[1]Department of Pathology, Lady Hardinge Medical College, New Delhi, India

[1 ]Department of Orthopedics, Lady Hardinge Medical College, New Delhi, India

Author notes

Address for correspondence: Prof. Manjula Jain, Department of Pathology, Lady Hardinge Medical College, New Delhi – 110 001, India. E-mail: dr_manjulajain@ 123456rediffmail.com

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Publisher ID: JPN-7-67

DOI: 10.4103/1817-1745.97631

PMC ID: 3401662

PubMed ID: 22837786

SO-VID: 90fa425d-8461-4e95-80b9-228e5d3f0adf

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This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Primary spinal intramedullary primitive neuroectodermal tumor

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Most cited references 19

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

The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors.

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

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