These include cancers that arise within peripheral nerves as well as plexiform neurofibromas that start out as benign tumors but then subsequently become cancerous. Nerve cancers are generally rapidly-growing and painful. It is important to operate on these lesions in a timely fashion before they have a chance to spread to other parts of the body. Once they metastasize, they are typically rapidly lethal. Goals of surgery for malignant nerve tumors are to obtain a tissue diagnosis, but not to necessarily remove the tumor. Although this seems counterintuitive to many people, attempting to remove the malignant tumor in the same fashion as removing a benign nerve tumore is essentially impossible. That is because cancer cells, invisible to the surgeons eyes, have already spread along the course of the affected nerve, even before the surgery is performed. Therefore just removing the tumor that the surgeon can see and feel will always leave some of the cancer behind.

Once these tumors are diagnosed by an open biopsy, a cure generally requires either limb amputation or at least removal of the entire adjacent nerve and all of the surrounding tissues, including muscle, blood vessels, and fat. Sometimes radiation and/or chemotherapy can be helpful, depending upon the exact type of tumor it turns out to be.