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A clinicopathological and immunohistochemical study of malignant peripheral nerve sheath tumors

*, Siny Vellukara Sasidharan and *, Vinu Kumar and Pai, Radha R. and *, Sheela Vasudevan (2014) A clinicopathological and immunohistochemical study of malignant peripheral nerve sheath tumors. International Journal of Research in Medical Sciences, 2 (4). pp. 1307-1312. ISSN 2320-6012

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Abstract

Background: Malignant Peripheral Nerve Sheath Tumor (MPNST) is a rare aggressive sarcoma that develops within a peripheral nerve and forms a diagnostic challenge in view of its varied histomorphology. This short series highlights the clinicopathological spectrum of 11 cases of MPNST and the incidence of neurofibromatosis 1 (NF1) association. Methods: This retrospective and descriptive study on MPNST was done in the department of pathology, Kasturba medical college Mangalore (Manipal University), India over a period of three years from January 2008 to December 2010. Cases which were histopathologically diagnosed as MPNST were reviewed & immunostains was done where ever indicated to rule out the differentials. Results: A total of 11 cases of MPNST were documented with a wide age range of 17-85 years. Male:female ratio was 2.6:1. Extremities (63.64%) were found to be the most common site. Location wise most of the tumors were deep seated (63.64%) and maximum cases were high grade (54.55%). NF1 association was seen in 2 cases. Heterologous elements in the form of chondroid differentiation was seen in one case. Immunostain with S-100 was focally positive in all the cases. Conclusion: MPNST is a highly aggressive sarcoma with poor prognosis characterized by a challenge in its diagnosis as it has several mimics. Its diagnosis necessitates the incorporation of clinicopathological features and IHC with S-100 protein.

Item Type: Article
Uncontrolled Keywords: Malignant peripheral nerve sheath tumor, Neurofibromatosis (NF-1), Heterologous differentiation
Subjects: Medicine > KMC Mangalore > Pathology
Depositing User: KMCMLR User
Date Deposited: 04 Dec 2014 09:56
Last Modified: 04 Dec 2014 09:56
URI: http://eprints.manipal.edu/id/eprint/141197

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