Morphological spectrum of peripheral nerve sheath tumors: A series of 126 cases

By convention, soft tissue tumors include the peripheral nerve sheath tumor (PNST) although they arise from neuroectoderm. PNSTs display a wide spectrum of morphology and biological behavior. Malignant transformation in benign tumors, especially neurofibroma, is an acknowledged phenomenon.This artic...

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Main Authors: Gabhane Sushma, Kotwal Mrunamayi, Bobhate Sudhakar
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2009-01-01
Series:Indian Journal of Pathology and Microbiology
Subjects:
Online Access:http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2009;volume=52;issue=1;spage=29;epage=33;aulast=Gabhane
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author Gabhane Sushma
Kotwal Mrunamayi
Bobhate Sudhakar
author_facet Gabhane Sushma
Kotwal Mrunamayi
Bobhate Sudhakar
author_sort Gabhane Sushma
collection DOAJ
description By convention, soft tissue tumors include the peripheral nerve sheath tumor (PNST) although they arise from neuroectoderm. PNSTs display a wide spectrum of morphology and biological behavior. Malignant transformation in benign tumors, especially neurofibroma, is an acknowledged phenomenon.This article describes clinico-pathological features of 126 PNSTs with their morphological variants and those associated with neurofibromatosis 1 (NF1) encountered in a single center over a 6-year period. Benign PNSTs were more common [118 (93.65%)] when compared with their malignant counterparts [8 (6.34%)]. Histological variants including plexiform schwannomas and diffuse, plexiform and pigmented forms of neurofibromas, though rare, were observed. Eleven (8.73%) patients with NF1 showed a strong association with plexiform neurofibroma. A malignant transformation in pre-existing neurofibroma was seen in 1 patient with NF1. This series highlights the clinico-pathological spectrum of PNSTs, their morphological variants and the incidence of associated NF1. The importance of detecting plexiform variants lies in their vastly different prognostic implications particularly when occurring in the setting of NF1
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spelling doaj.art-2b73da10a03f4aa28b9bcf51073be84f2022-12-21T19:55:46ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292009-01-015212933Morphological spectrum of peripheral nerve sheath tumors: A series of 126 casesGabhane SushmaKotwal MrunamayiBobhate SudhakarBy convention, soft tissue tumors include the peripheral nerve sheath tumor (PNST) although they arise from neuroectoderm. PNSTs display a wide spectrum of morphology and biological behavior. Malignant transformation in benign tumors, especially neurofibroma, is an acknowledged phenomenon.This article describes clinico-pathological features of 126 PNSTs with their morphological variants and those associated with neurofibromatosis 1 (NF1) encountered in a single center over a 6-year period. Benign PNSTs were more common [118 (93.65%)] when compared with their malignant counterparts [8 (6.34%)]. Histological variants including plexiform schwannomas and diffuse, plexiform and pigmented forms of neurofibromas, though rare, were observed. Eleven (8.73%) patients with NF1 showed a strong association with plexiform neurofibroma. A malignant transformation in pre-existing neurofibroma was seen in 1 patient with NF1. This series highlights the clinico-pathological spectrum of PNSTs, their morphological variants and the incidence of associated NF1. The importance of detecting plexiform variants lies in their vastly different prognostic implications particularly when occurring in the setting of NF1http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2009;volume=52;issue=1;spage=29;epage=33;aulast=GabhaneMPNSTneurofibromaneurofibromatosis 1peripheral nerve sheath tumorschwannoma
spellingShingle Gabhane Sushma
Kotwal Mrunamayi
Bobhate Sudhakar
Morphological spectrum of peripheral nerve sheath tumors: A series of 126 cases
Indian Journal of Pathology and Microbiology
MPNST
neurofibroma
neurofibromatosis 1
peripheral nerve sheath tumor
schwannoma
title Morphological spectrum of peripheral nerve sheath tumors: A series of 126 cases
title_full Morphological spectrum of peripheral nerve sheath tumors: A series of 126 cases
title_fullStr Morphological spectrum of peripheral nerve sheath tumors: A series of 126 cases
title_full_unstemmed Morphological spectrum of peripheral nerve sheath tumors: A series of 126 cases
title_short Morphological spectrum of peripheral nerve sheath tumors: A series of 126 cases
title_sort morphological spectrum of peripheral nerve sheath tumors a series of 126 cases
topic MPNST
neurofibroma
neurofibromatosis 1
peripheral nerve sheath tumor
schwannoma
url http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2009;volume=52;issue=1;spage=29;epage=33;aulast=Gabhane
work_keys_str_mv AT gabhanesushma morphologicalspectrumofperipheralnervesheathtumorsaseriesof126cases
AT kotwalmrunamayi morphologicalspectrumofperipheralnervesheathtumorsaseriesof126cases
AT bobhatesudhakar morphologicalspectrumofperipheralnervesheathtumorsaseriesof126cases