Malignant peripheral nerve sheath tumors are cancerous growths in the soft tissue lining of a nerve, often caused by neurofibromatosis. Symptoms include pain, weakness, and fatigue. Treatment may involve surgery, chemotherapy, and radiation, with a good prognosis if caught early.
A malignant peripheral nerve sheath tumor is a cancerous growth that appears in the soft tissue lining of a nerve. It most commonly develops around a long nerve in one of the extremities, although a tumor can also affect a nerve in the chest, abdomen, pelvis, or neck. A person may experience pain and weakness in the area of the growth that gradually gets worse over time. A small, malignant peripheral nerve sheath tumor can often be removed by surgery, but large or multiple tumors may require a combination of surgery, chemotherapy, and radiation in an effort to shrink them and reduce the chances of the cancer spreading to other parts of the body.
Most people who develop nerve sheath tumors have an underlying genetic disorder called neurofibromatosis. The disorder predisposes an individual to nerve problems, including tissue scarring and tumors, although the condition is usually benign. Only a small number of patients develop malignancies. If a malignant peripheral nerve sheath tumor is present but neurofibromatosis is not, the underlying cause is usually never determined. Nerve sheath tumors can affect a person of any age, although they are more commonly diagnosed in adult males.
A new, small, malignant peripheral nerve sheath tumor may not cause any obvious symptoms. As the mass grows, it can begin to cause occasional sharp pains, tingling sensations, and body aches in the region. It may be possible to feel or see a tumor if it grows close to the skin. Symptoms of fatigue and chronic muscle weakness can be indicative of an advanced stage cancer that is starting to metastasize.
A doctor can diagnose a nerve sheath tumor by asking about symptoms, doing genetic testing for neurofibromatosis, and taking imaging scans of the mass. A tissue biopsy is also usually needed to see if the growth is cancerous or benign. Doctors determine the best course of treatment based on the size, stage, and location of the malignant peripheral nerve sheath tumor.
Small growths on the extremities and easily accessible sites in the trunk and neck can often be removed by a surgeon who specializes in this type of surgery. Extreme precautions are taken during the surgery to avoid damaging the delicate underlying nerve tissue. Surgery has a high success rate, but accidental damage could lead to temporary loss of sensation or permanent paralysis in one part of the body. Radiation treatments and chemotherapy can be helpful in fighting intermediate- and late-stage nerve sheath tumors. The prognosis is generally good when patients receive early and aggressive treatment.
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