A malignant tumor that arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei, hypocellular areas, and areas featuring organized whorls of fibroblastic proliferation. The most common primary sites are the extremities, retroperitoneum, and trunk. These tumors tend to present in childhood, often in association with NEUROFIBROMATOSIS 1. (From DeVita et al., Cancer: Principles & Practice of Oncology, 5th ed, p1662; Mayo Clin Proc 1990 Feb;65(2):164-72)

A malignant neurilemmoma with nerve sheath differentiation. It is often associated with NEUROFIBROMATOSIS 1 and RHABDOMYOSARCOMA.

Synonyms: Neurilemmosarcomas, Neurilemmoma, Malignant, Malignant Neurilemomas, Sarcomas, Neurogenic, Neurofibrosarcomas, Neurogenic Sarcoma, Neurogenic Sarcomas, Schwannoma, Malignant, Malignant Neurilemmoma, Peripheral Nerve Sheath Tumors, Malignant, Malignant Schwannomas, Neurilemoma, Malignant, MPNST, Malignant Neurilemmomas, Malignant Peripheral Nerve Sheath Tumor, Neurilemmosarcoma, Malignant Neurilemoma, Malignant Schwannoma, Malignant Peripheral Nerve Sheath Tumors, Sarcoma, Neurogenic, Neurofibrosarcoma