• Peripheral Nerve Sheath Tumor (Neurofibrosarcoma)

    Peripheral nerve sheath tumors, also called neurofibrosarcomas, are malignant tumors that form in the soft tissues surrounding the peripheral nerves, which receive messages from the brain and stimulate voluntary movement. These tumors are usually found in the arms and legs and can spread extensively along nerve tissue.

    Perifpheral Nerve Sheath Tumors Treatment at Dana-Farber/Boston Children's

    Patients with peripheral nerve sheath tumors are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Bone & Soft Tissue Program. Continue reading to learn more about peripheral nerve sheath tumors or visit the Bone & Soft Tissue Program homepage to learn about our expertise.

    Symptoms & Diagnosis

    Children with neurofibromatosis type 1 are at very high risk of developing these tumors. Because they affect tissue that is elastic and easily moved, peripheral nerve sheath tumors may exist for a long time before they are diagnosed. The most common symptoms include:

    • Painless swelling or a lump, usually in the arms or legs
    • Pain or soreness from compressed nerves or muscles
    • Nerve loss as the tumor grows
    • Limp or difficulty using the arms, legs, feet or hands

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

    • Biopsy
    • X-ray
    • Magnetic resonance imaging (MRI)
    • Computerized tomography (CT or CAT) scan
    • Bone scan or PET scan
    • Blood tests, including a complete blood count

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

    Treatment & Care Options

    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.

    Progressive or Recurrent Disease

    It is possible for a peripheral nerve sheath tumor to recur, even after aggressive treatment.

    Long-term Outlook

    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% if the tumor is small and resected, to less than 30% if it has spread to other organs.