Malignant peripheral nerve sheath tumors are rare, high-grade, soft-tissue sarcoma that typically arise from Schwann cells and typically occur in adults (more so than children).
They are locally invasive and often present as an enlarging mass.
They can arise from malignant transformation of a plexiform neurofibroma, thus they have a strong association with Neurofibromatosis, Type 1.
References
Adesina, Adekunle M., Tarik Tihan, Christine E. Fuller, and Tina Young Poussaint. Atlas of Pediatric Brain Tumors. Springer, 2016.
Bockenheimer, Ph (Philipp). Atlas of Clinical Surgery; with Special Reference to Diagnosis and Treatment for Practitioners and Students. New York?: Rebman, 1908. http://archive.org/details/atlasofclinicals00bock.
Gray, Frangoise, Charles Duyckaerts, and Umberto De Girolami. Escourolle and Poirier’s Manual of Basic Neuropathology. OUP USA, 2013.
Newton, Herbert B. Handbook of Brain Tumor Chemotherapy, Molecular Therapeutics, and Immunotherapy. Academic Press, 2018.
Orkin, Stuart H., David E. Fisher, A. Thomas Look, Samuel Lux, David Ginsburg, and David G. Nathan. Oncology of Infancy and Childhood E-Book. Elsevier Health Sciences, 2009.
Image Reference
Malignant peripheral nerve sheath tumor of left tibia
“Cases Journal.” Cases Journal. Accessed July 16, 2018. https://casesjournal.biomedcentral.com/.