Malignant peripheral nerve sheath tumors (MPNST) are soft tissue tumors showing neuroectodermal differentiation. They arise in connection to a major nerve trunk, a peripheral nerve or a preexisting neuroma in patients with hereditary neurofibromatosis type 1. [1, 2]
They usually develop in the deep soft tissue between anatomic compartments, the root of the limbs (inguinal region, the axilla) being one of the most common sites of occurrence.