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An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists

Year : 2020  |  Volume : 24  |  Issue : 4  |  Page : 86-90
Malignant peripheral nerve sheath tumor: A rare malignancy

1 Department of OMR, AIIMS, New Delhi, India
2 Department of OMR, GDCH, Ahmedabad, Gujarat, India
3 Department of OMR, Dhulikhel Hospital, Kathmandu University, School of Medical Sciences, Nepal

Correspondence Address:
Sheetal Sharma
G-7 Leela Homes, Vaishali, Ghaziabad, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jomfp.JOMFP_9_20

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Malignant peripheral nerve sheath tumor (MPNST) is also termed as spindle cell malignancy of the peripheral nerve Schwann cell. It is a rare and highly aggressive, soft-tissue sarcoma of ectomesenchymal origin that accounts for 10% of all sarcomas and only 10%–12% of all lesions occur in the head-and-neck region, thus making it a rare entity. It arises de novo or from the preexisting benign neurofibroma. The diagnosis of MPNST is one of the most elusive among the soft-tissue tumors because of its greater variability in overall presentation both clinically and histologically. This difficulty can be overcome by the use of immunohistochemistry. This article presents a rare case of MPNST of the oral cavity in a 40-year-old female patient with a brief review of the current literature.

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Journal of Oral and Maxillofacial Pathology | Published by Wolters Kluwer - Medknow
Online since 15th Aug, 2007