Malignant Peripheral Nerve Sheath Tumor

What is a Malignant Peripheral Nerve Sheath Tumor?

Malignant peripheral nerve sheath tumors are a rare form of sarcoma that forms in the protective linings surrounded the major nerves of the body. This is an aggressive form of cancer that is hard to treat due to the location of the tumors making surgery difficult and limited how much medication reaches the affected area.

While many tumors that form in the nervous system are benign, these tumors are malignant and will continue to grow and spread without treatment. Most cases are caused by a genetic disorder, radiation exposure, or unknown triggers.

What are the Symptoms of a Malignant Peripheral Nerve Sheath Tumor?

Since the tumors are hidden deep in the peripheral nerves, there are often no early symptoms. As the tumors grow and press on the nerves, patients begin to notice pain and numbness in the affected area.

The peripheral nerves connect to the limbs, so pain can be felt almost anywhere in the limbs or torso. Some patients notice a palpable mass in their muscle tissue, or develop weakness and dizziness due to nerve compression.

How is a Malignant Peripheral Nerve Sheath Tumor Treated?

Since this form of cancer is hard to treat and aggressive, patients need multiple specialists working together on their case. Oncologists with specific training and experience in sarcoma management plan out surgeries to remove as much of the tumor or tumors as possible, following with chemotherapy to target any remaining material.

Nerve and pain specialists help limit damage to the body’s functions and assist the patient in recovering. Genetic testing and therapy can also help the patient determine what caused the cancer to form and what other complications may arise due to their genetic makeup. Relapse rates are high with this form of sarcoma, so ongoing monitoring is necessary for the rest of the patient’s life.

Last Reviewed:
October 10, 2016
Last Updated:
August 09, 2017
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