Surgery is the mainstay of treatment for malignant peripheral nerve sheath tumor. For patients suffering from neurofibrosarcomas in an extremity, if the tumor is vascularized and has many nerves going through it and/or around it, amputation of the extremity may be necessary. Some surgeons argue that amputation should be the procedure of choice when possible, due to the increased chance of a better quality of life. Otherwise, surgeons may opt for a limb-saving treatment, by removing less of the surrounding tissue or part of the bone, which is replaced by a metal rod or grafts.

• The first-line treatment is surgical resection with wide margins. The removal of the tumor along with surrounding tissue may be vital for the patient’s survival.
• For discrete, localized tumors, surgery is often followed by radiation therapy of the excised area to reduce the chance of recurrence.
• For patients suffering from neurofibrosarcomas in an extremity, if the tumor is vascularized and has many nerves going through it and/or around it, amputation of the extremity may be necessary. Some surgeons argue that amputation should be the procedure of choice when possible, due to the increased chance of a better quality of life. Otherwise, surgeons may opt for a limb-saving treatment, by removing less of the surrounding tissue or part of the bone, which is replaced by a metal rod or grafts.