Symptoms may include: Soft tissue sarcomas have been linked within families, so it is hypothesized that neurofibrosarcoma may be genetic, although researchers still do not know the exact cause of the disease.

The p53 (a tumor suppressor gene in the normal population) genome on 17p in neurofibrosarcoma patients is mutated, increasing the probability of cancer.

A malignant peripheral nerve sheath tumor is rare, but is one of the most common frequent soft tissue sarcoma in the pediatric population.

About half of these cases also happen to occur along with neurofibromatosis type 1 (NF-1), which is a genetic mutation on the 17th chromosome which causes tumors along the nervous system.

For discrete, localized tumors, surgery is often followed by radiation therapy of the excised area to reduce the chance of recurrence.

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.

Features associated with poor prognosis include a large primary tumor (over 5 cm across), high grade disease, co-existent neurofibromatosis, and the presence of metastases.