Malignant Peripheral Nerve Sheath Tumor

Also known as: MPNST, neurofibrosarcomas

What are malignant peripheral nerve sheath tumors?

The peripheral nerves are the nerves found in the body aside from the central nervous system (brain and spinal cord). When the sheath that protects these nerves develops cancer in its cells, this is known as malignant peripheral nerve sheath tumors, or MPNST.

What causes malignant peripheral nerve sheath tumors?

Like most forms of cancer, malignant peripheral nerve sheath tumors develop due to changes in a cell’s DNA. What specifically causes these changes to occur isn’t clear. People with certain genetic conditions, such as neurofibromatosis type 1, are more likely to develop malignant peripheral nerve sheath tumors.

What are the symptoms of malignant peripheral nerve sheath tumors?

Possible symptoms of malignant peripheral nerve sheath tumors include a growing lump of tissue beneath the skin, pain where the tumor is growing and weakness in that part of the body. Symptoms tend to develop and then get worse quickly with malignant peripheral nerve sheath tumors.

How can malignant peripheral nerve sheath tumors affect children?

Malignant peripheral nerve sheath tumors are rare but can develop in children as well as adults. Children with neurofibromatosis type 1 are at a high risk of developing the condition.

What are malignant peripheral nerve sheath tumor treatments?

Surgery to remove the tumor and surrounding tissue, radiation therapy and chemotherapy are all potential treatments for malignant peripheral nerve sheath tumors. In many cases, these treatments are used in combination to rid the body of cancer.

Reviewed by: Maggie Fader, MD

This page was last updated on: November 03, 2023 12:16 PM