Primary peripheral primitive neuroectodermal tumor of the mandible

Primitive neuroectodermal tumor (PNET) is a broad term that includes a wide array of lesions with varying differentiating potential affecting both the central and peripheral nervous system. Peripheral PNET (pPNET), a variant of PNET, is known to arise in many places throughout the body; involvement...

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Main Authors:	Krishnamurthy, Arvind, Ramshankar, Vijayalakshmi, Majhi, Urmila, George, Rinku
Format:	Online
Language:	English
Published:	Medknow Publications & Media Pvt Ltd 2013
Online Access:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3814671/

id	pubmed-3814671
recordtype	oai_dc
spelling	pubmed-38146712013-11-07 Primary peripheral primitive neuroectodermal tumor of the mandible Krishnamurthy, Arvind Ramshankar, Vijayalakshmi Majhi, Urmila George, Rinku Case Report - Surgical Pathology Primitive neuroectodermal tumor (PNET) is a broad term that includes a wide array of lesions with varying differentiating potential affecting both the central and peripheral nervous system. Peripheral PNET (pPNET), a variant of PNET, is known to arise in many places throughout the body; involvement of the mandible is however rare. A review of the scientific literature describes only seven reports of pPNET to be arising from the mandible. Given their insidious clinical symptoms, variable locations and rarity, the accurate diagnosis of peripheral PNETs poses a challenge for clinicians. PNETs exhibits characteristic immunophenotypical and genetic features that distinguish them from other small round cell tumors. A multidisciplinary approach is necessary to manage patients affected by PNET. There is however no consensus about the best therapeutic strategy. We recently got to treat a young patient with mandibular PNET; the clinical course as well as the histopathology with immunohistochemistry correlation of this rare entity is discussed. Medknow Publications & Media Pvt Ltd 2013 /pmc/articles/PMC3814671/ /pubmed/24205482 http://dx.doi.org/10.4103/2231-0746.119219 Text en Copyright: © Annals of Maxillofacial Surgery http://creativecommons.org/licenses/by-nc-sa/3.0 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.
repository_type	Open Access Journal
institution_category	Foreign Institution
institution	US National Center for Biotechnology Information
building	NCBI PubMed
collection	Online Access
language	English
format	Online
author	Krishnamurthy, Arvind Ramshankar, Vijayalakshmi Majhi, Urmila George, Rinku
spellingShingle	Krishnamurthy, Arvind Ramshankar, Vijayalakshmi Majhi, Urmila George, Rinku Primary peripheral primitive neuroectodermal tumor of the mandible
author_facet	Krishnamurthy, Arvind Ramshankar, Vijayalakshmi Majhi, Urmila George, Rinku
author_sort	Krishnamurthy, Arvind
title	Primary peripheral primitive neuroectodermal tumor of the mandible
title_short	Primary peripheral primitive neuroectodermal tumor of the mandible
title_full	Primary peripheral primitive neuroectodermal tumor of the mandible
title_fullStr	Primary peripheral primitive neuroectodermal tumor of the mandible
title_full_unstemmed	Primary peripheral primitive neuroectodermal tumor of the mandible
title_sort	primary peripheral primitive neuroectodermal tumor of the mandible
description	Primitive neuroectodermal tumor (PNET) is a broad term that includes a wide array of lesions with varying differentiating potential affecting both the central and peripheral nervous system. Peripheral PNET (pPNET), a variant of PNET, is known to arise in many places throughout the body; involvement of the mandible is however rare. A review of the scientific literature describes only seven reports of pPNET to be arising from the mandible. Given their insidious clinical symptoms, variable locations and rarity, the accurate diagnosis of peripheral PNETs poses a challenge for clinicians. PNETs exhibits characteristic immunophenotypical and genetic features that distinguish them from other small round cell tumors. A multidisciplinary approach is necessary to manage patients affected by PNET. There is however no consensus about the best therapeutic strategy. We recently got to treat a young patient with mandibular PNET; the clinical course as well as the histopathology with immunohistochemistry correlation of this rare entity is discussed.
publisher	Medknow Publications & Media Pvt Ltd
publishDate	2013
url	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3814671/
_version_	1612022496801849344

Primary peripheral primitive neuroectodermal tumor of the mandible

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