Malignant peripheral nerve sheath tumors
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Malignant peripheral nerve sheath tumors are the tumors that form in the lining of the nerves that extend from the spinal cord into the body. It can be found in any part of the body, especially the deep tissue of the arms, legs, and trunk. This rare type of cancer can cause the affected area to be painful and weak or have a lump that grows bigger over time.
Surgery is mostly recommended for malignant peripheral nerve sheath tumors, but radiation therapy and chemotherapy can also be alternatives in certain cases.
Symptoms
Signs and symptoms of malignant peripheral nerve sheath tumors include:
- Constant pain
- Weakness
- Lump of tissue under skin
In case of persistent signs and symptoms, seeing a doctor is recommended.
Causes
Causes of malignant peripheral nerve sheath tumors are still unclear. All that is known for now is that the cancerous cells originate from genetic mutations happening in the protective lining around a nerve. This abnormality causes the affected cells to multiply out of control without dying, unlike normal cells. This leads to accumulations of abnormal cells which form a tumor that can spread to nearby tissue or any other parts of the body.
Risk factors
Contributing factors of malignant peripheral nerve sheath tumors include:
- History of radiation therapy: to treat cancer, putting the radiated area at risk of formation of a malignant peripheral nerve sheath tumor in 10-20 years after treatment.
- Noncancerous nerve tumors: such as neurofibroma, as they can become an original site of malignant peripheral nerve sheath tumors.
- Inherited condition that increases risk of nerve tumors: such as Neurofibromatosis type 1.
Diagnosis
Diagnosing malignant peripheral nerve sheath tumors can be done in many ways, including:
- Neurological examination: which helps the doctor have a better understanding of your symptoms and plan for diagnosis.
- Imaging tests: to help your doctor determine the size of the tumor and if it has spread to any other parts of the body including a Magnetic resonance imaging (MRI), magnetic resonance neurography, Computed tomography (CT) and positron emission tomography (PET).
- Biopsy: a process where your specialist removes a sample of tissue for specialized testing in a laboratory before or during surgery. Your doctor will use the testing results to predict the progression of the disease and choose the best treatment.
Treatment
Common treatments for malignant peripheral nerve sheath tumors include:
Surgery
Where the surgeon aims to remove the entire or part of the tumor together with a certain amount of the margin of surrounding non-cancerous tissue. Surgery poses a varying risk of nerve damage and disability, depending on the location and size of the malignant tumor. If the tumors occurring in the arms and legs, it may be necessary to have limb amputation. Doing radiation before surgery to shrink a tumor can increase the success rate of removing the entire tumor in some cases.
Radiation therapy
Which uses high-energy beams, such as X-rays and protons, to shrink a tumor by killing its cancer cells before surgery. This will help to increase the success of the surgery. Radiation therapy is also useful in killing remaining cancer cells after surgery.
Chemotherapy
Which helps control your symptoms and slow the cancer’s growth using drugs to kill cancer cells. This may be recommended to patients whose cancer has already spread to other parts of the body.
Rehabilitation
Which is a postoperative care where physical therapists and occupational therapists help a patient recover lost function and mobility due to nerve damage or limb amputation.
