Open AccessMalignant peripheral nerve sheath tumor: A rare malignancy
Author(s) -
Sheetal Sharma,
Jigna S Shah,
Harleen Bali
Publication year - 2020
Publication title -
journal of oral and maxillofacial pathology/journal of oral and maxillofacial pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.455
H-Index - 25
eISSN - 1998-393X
pISSN - 0973-029X
DOI - 10.4103/jomfp.jomfp_9_20
Subject(s) - malignant peripheral nerve sheath tumor , malignancy , medicine , peripheral nerve , pathology , peripheral , anatomy , neurofibromatosis
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.