Sandeep Kumar Kar, Tanmoy Ganguly and Swarnali Dasgupta
Malignant peripheral nerve sheath tumour (MPNST) is a rare and very aggressive tumour of nerve cell origin associated with poor prognosis. Incidence of MPNST is 1 per 1, 00,000 populations and it constitutes between 3%-10% of all soft tissue sarcomas1-4. MPNST have been found to be associated with neurofibromatosis type 1 (associated with mutation in NF-1 gene) in 2%-29% cases . Male and female are almost equally (53:47) involve. These tumours often create diagnostic dilemmas because of non-specific clinical diagnostic criteria, histopathological resemblance with other spindle cell sarcomas like monophasic synovial sarcoma, leiomyosarcoma and fibrosarcoma.
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