Malignant peripheral nerve sheath tumor — from genetics to multidisciplinary treatment
Anna M. Czarnecka,
Paulina Chmiel,
Paweł Sobczuk
et al.
Abstract:Malignant peripheral nerve sheath tumor (MPNST) is an aggressive soft tissue sarcoma (STS); it originates from nervous tissue and typically develops in proximity to nerve trunks in the limbs and trunk. These tumors, constituting approximately 5% of soft tissue sarcomas, can either form spontaneously or arise from pre-existing neurofibromas. The majority (90%) of cases occur in individuals between the 2 nd and 5 th decades of life. The main risk factor for MPNST is von Recklinghausen disease (type 1 neurofibrom… Show more
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