Neurofibrosarcoma

What is 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.

What are the symptoms of neurofibrosarcoma?

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
  • pain or soreness from compressed nerves or muscles
  • nerve loss
  • limp or difficulty using the arms, legs, feet or hands

What causes neurofibrosarcoma?

The exact cause of neurofibrosarcoma is not entirely understood, however studies have indicated that genetics may play a role in the formation of all soft tissue sarcomas. Limited studies have shown a possible link between soft tissue sarcomas and other types of cancer.

Who is at increased risk for developing neurofibrosarcomas?

If your child has neurofibromatosis (which involves alterations in the NF1 gene), he may be at an increased risk for developing neurofibrosarcoma. These tumors occur in up to 16 percent of people with neurofibromatosis, also known as Recklinghausen's disease. This is a genetic condition in which benign fibrous tumors develop inside nerve tissue.

How we care for neurofibrosarcoma

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.