Peripheral nerve sheath tumor (neurofibrosarcoma)
How common are neurofibrosarcomas?
Neurofibrosarcoma is considered a soft tissue sarcoma—a cancer that originates in soft tissue, which includes fat, muscles, tendons, nerves, synovial tissue, blood vessels and other fibrous tissue.
- As a group, soft tissue sarcomas account for less than 1 percent of all new cancer cases each year. In the United States, about 900 children and adolescents are diagnosed with soft tissue sarcomas each year.
- Neurofibrosarcoma accounts for 5 to 7 percent of all soft tissue sarcoma cases.
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. Neurofibromatosis, also known as Recklinghausen's disease, is a genetic (inherited) condition in which benign fibrous tumors develop inside nerve tissue.
What are the symptoms of neurofibrosarcoma?
Since neurofibrosarcoma affects tissue that is elastic and easily moved around instead of hard and stationary, a tumor may exist for a long time before being discovered, growing large and pushing aside surrounding tissue.
While each child may experience symptoms differently, and symptoms can vary greatly depending on size, location, and spread of tumor, some of the most common include:
- a painless swelling or lump, usually in the arms or legs
- pain or soreness
- limping or other difficulty using the arms, legs, feet or hands
The symptoms of neurofibrosarcoma may resemble other conditions or medical problems. Always consult a physician for a diagnosis.
What is the long-term outlook for patients with neurofibrosarcoma?
Your child’s prognosis greatly depends on:
- the extent of the disease
- the size and location of the tumor
- a presence of absence of metastasis
- the tumor's response to therapy
- the age and overall health of your child
- your child’s tolerance of specific medications, procedures, or therapies
- new developments in treatment
As with any cancer, prognosis and long-term survival can vary greatly. Prompt medical attention and aggressive therapy are important for the best outcome. Because side effects of radiation and chemotherapy, as well as recurrence of the disease, may occur, continuous follow-up care is essential.