Recurrent MPNST in Mosaic Localized Neurofibromatosis: A Rare Scenario – Review

Malignant peripheral nerve sheath tumor (MPNST) is a rare spindle cell neoplasm accounting for approximately 3-5% of soft tissue sarcomas. It often arises from a peripheral nerve, from a pre-existing benign nerve sheath tumor, or in a patient with neurofibromatosis type 1(NF1). In the absence of these settings, particularly in sporadic or radiation associated tumors, the diagnosis can be more challenging and is based on the histological and immunohistochemical features suggesting Schwannian differentiation