Neurofibrosarcoma | Diagnosis & Treatment

How is neurofibrosarcoma diagnosed?

In addition to a complete physical exam, a doctor may order the following to diagnose a peripheral nerve sheath tumor:

After all tests are completed, doctors will be able to outline the best treatment options.


Once neurofibrosarcoma has been diagnosed, the tumor is given a “stage,” usually stage I, stage II, stage III or stage IV. This indicates how far the tumor has spread from its original location. The stage helps doctors decide which form of treatment is most appropriate, and predicts how the condition is likely to respond to therapy.

Neurofibrosarcoma may be localized (meaning it has not spread beyond the nerve tissue where it arose) or metastatic (meaning it has spread, in this case usually to the lungs). However, most often, neurofibrosarcoma remains localized.

What are the treatment options for neurofibrosarcoma?

Surgery to remove the entire tumor and nearby tissue is the standard treatment for peripheral nerve sheath tumors. Depending on the size and location of the tumor, it may be necessary to remove all or part of a limb. In most cases, a limb-sparing procedure is used to avoid amputation. Chemotherapy and radiation therapy may be given before surgery to shrink the tumor or after surgery to kill any remaining cancer cells.

What is the long-term outlook for children with neurofibrosarcoma?

The prognosis is directly related to the size and extension of the tumor and the ability to completely resect it with surgery. The 5-year survival rates range from close to 80 percent if the tumor is small and resected, to less than 30 percent if it has spread to other organs.