Malignant Peripheral Nerve Sheath Tumors – Symptoms and Causes
Overview
Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon cancers. They develop in the protective covering of peripheral nerves—the nerves extending from the spinal cord to other body parts.
Previously known as neurofibrosarcomas, these tumors can appear anywhere in the body.
MPNSTs most commonly affect the deep tissues of the arms, legs, and trunk. People with these tumors typically experience pain and weakness in the affected area. A noticeable growing lump or mass might also develop over time.
Signs and Problems
People with nerve sheath tumors often notice symptoms that worsen rapidly. You might experience:
- Pain in the area where the tumor is growing
- Muscle weakness in the affected body part
- A noticeable lump that grows under the skin
These symptoms can interfere with daily activities and may become more severe over time. The pain might begin as mild discomfort but can intensify as the tumor enlarges.
When You Should Contact a Healthcare Provider
If you notice any persistent symptoms like unexplained pain, growing lumps, or weakness that doesn’t go away, schedule an appointment with your doctor.
Since these nerve tumors are uncommon, your healthcare provider will likely check for more common causes of your symptoms first.
Don’t delay seeking medical attention if you experience:
- Severe pain that disrupts sleep or daily activities
- Rapid growth of a lump under your skin
- Progressive weakness in any part of your body
Your doctor might recommend imaging tests like MRI scans to examine any suspicious areas more closely.
Causes
The exact triggers for most malignant peripheral nerve sheath tumors remain unknown. These cancers develop when DNA changes occur in cells that surround nerves.
DNA contains the instructions for cell function, and these alterations tell the cells to multiply rapidly and avoid the normal cell death process.
When these abnormal cells accumulate, they form a tumor. This growth can invade nearby healthy tissue and destroy it. If left unchecked, these cancer cells may eventually spread to distant parts of the body.
Risk Factors
Several factors can increase a person’s chances of developing malignant peripheral nerve sheath tumors:
- Previous Radiation Treatment: These tumors may develop in areas that received radiation therapy 10-20 years earlier.
- Existing Benign Nerve Tumors: Noncancerous nerve tumors like neurofibromas can sometimes transform into malignant tumors.
- Genetic Conditions: People with neurofibromatosis 1, an inherited disorder, face a higher risk of developing these tumors.
