Benign nerve sheath tumors sometimes do not require treatment beyond monitoring them to make sure they don’t grow or spread. Malignant peripheral nerve sheath tumors, however, offer a poorer prognosis, especially if they start to spread. These tumors are prone to recurrence after treatment and distant metastasis, meaning the spread all over the body. Once these tumors metastasize, they are often quickly lethal. Tumors that stem from neurofibromatosis type 1 typically offer lower chances of surviving than sporadic malignant tumors.
After diagnosis, malignant tumors can require a limb amputation or the removal of the entire adjacent nerve and all of the surrounding tissues.