Malignant Peripheral Nerve Sheath Tumor (MPNST)
What are malignant peripheral nerve sheath tumors?
Malignant peripheral nerve sheath tumors are sarcomas that develop within the protective lining of a nerve. This type of tumor is very rare.
What causes malignant peripheral nerve sheath tumors?
Like other cancers, a malignant peripheral nerve sheath tumor is due to a mutation in the DNA that causes cells within the nerve sheath to rapidly multiply.
Who is at risk for a malignant peripheral nerve sheath tumor?
- Personal or family history of neurofibromatosis 1 (NF-1)
- Previous radiation therapy for cancer
What are the symptoms of a malignant peripheral nerve sheath tumor?
Depending on the nerve that the tumor is within, there is often pain in the affected area, weakness, and at times a palpable mass under the skin.
How is a malignant peripheral nerve sheath tumor diagnosed?
- Patient history
- Physical exam
- Imaging: CT, MRI, PET
- Biopsy: a biopsy is the only definitive way to diagnose a tumor
How is a malignant peripheral nerve sheath tumor treated?
In most cases, both nonsurgical and surgical methods are used in conjunction.
Nonsurgical
Chemo and radiation therapies
Surgical
Surgically remove the entire tumor