Malignant peripheral nerve sheath tumors can grow quickly and spread rapidly, and once they spread, they are usually lethal. This, surgery soon after diagnosis is imperative to stop the tumors from metastasizing. However, unlike other cancer surgeries, the goal in this case is not necessarily to remove the tumor—though surgeons will generally take out as much as possible while minimizing damage to nearby nerve and muscle tissue—but rather to obtain a tissue sample that can be used to accurately the tumor. Malignant tumors have often already spread throughout the affected nerve, so it is more or less impossible for the surgeon to remove all of them. Instead, after a surgical biopsy, treatment options sometimes involve amputating the entire limb or removing the entire adjacent nerve and the surrounding tissue, including muscle, blood vessels and fat.
Radiation therapy and chemotherapy may also be of some help, before, during or after surgery. Following surgery, patients may also need to undergo rehabilitation to restore function and feeling in the muscles controlled by the nerves from which the tumor was removed.