Neurofibrosarcoma

Also known as: peripheral nerve sheath tumor, soft tissue sarcoma

What is neurofibrosarcoma?

Peripheral nerves carry signals to and from the brain and spinal cord, they have a protective sheath that surrounds them. Neurofibrosarcoma (peripheral nerve sheath tumor) is a rare type of malignant cancer (sarcoma) in children that develops in the cells of the sheath of the arms and legs, spreading along the nerves.

What causes neurofibrosarcoma?

The cause of neurofibrosarcoma is unknown, however nerve cell tumors are more common in persons who have the inherited disorder called neurofibromatosis type 1.

What are the symptoms of neurofibrosarcoma?

Neurofibrosarcoma often doesn’t present with any symptoms until it’s been present for quite a long time. Common symptoms include a painless swelling or visible lump, pain or limping from compressed nerves or muscles, or other difficulties using the arms, legs, feet or hands.

What are neurofibrosarcoma care options?

Depending on the size and location of the tumor, treatments may include surgical removal (amputation of a part or a whole limb may be required), radiation and chemotherapy.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: September 16, 2025 12:30 PM