What Is a Childhood Peripheral Nerve Sheath Tumor?
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 often grow in the arms and legs.
- They can spread extensively along the nerve tissue.
At Dana-Farber/Boston Children's Cancer and Blood Disorders Center, your child will receive care from leading cancer center doctors and world-renowned pediatric experts. Specialists in our Bone and Soft Tissue Tumors Program have extensive experience treating children with peripheral nerve sheath tumors.
Causes and Symptoms of Childhood Peripheral Nerve Sheath Tumor
Children with neurofibromatosis type 1 are at very high risk of developing these neurofibrosarcoma tumors. It can be hard to discover and diagnose these types of tumors because they affect elastic and easily moved tissue.
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
How We Diagnose Childhood Peripheral Nerve Sheath Tumor
A doctor will complete a complete physical examination and order a variety of diagnostic tests. Testing may include sophisticated imaging studies, biopsies, and blood tests. After completing all necessary tests, our specialists will discuss the findings and recommended treatment plan with your family.
How We Treat Childhood Peripheral Nerve Sheath Tumor
We typically perform surgery to remove the tumor and nearby tissue to treat peripheral nerve sheath tumors. In most cases, our doctors use limb-salvage surgery to preserve as much of the limb and limb function as possible.
We may also treat patients with chemotherapy and radiation therapy before or after surgery, depending on the child's individual needs. Your medical team will discuss every step of treatment with you and your child.
After treatment, children treated for peripheral nerve sheath tumors continue to receive care through our pediatric cancer survivorship programs. Continued monitoring and care are essential as these tumors can recur, even after aggressive treatment.
Long-term Outcomes for Children with Peripheral Nerve Sheath Tumor
The prognosis depends on the size and extent of the tumor, and whether it's possible to remove it with surgery. The 5-year survival rates range from close to 80 percent if the tumor is small and removed, to less than 30 percent if it has spread to other organs.
Childhood Peripheral Nerve Sheath Tumor Treatment Team
Your child's care team will include a varied group of medical specialists who are experts in their fields of oncology, radiation therapy, surgery, and more. See a complete list of the specialists in our Childhood Solid Tumor Center.