Description

Malignant peripheral nerve sheath tumors are rare cancers that begin in the lining of the nerves. These cancers occur in the nerves that extend from the spinal cord in the body, called peripheral nerves. Malignant peripheral nerve sheath tumors that used to be called neurofibrosarcomas.

Malignant peripheral nerve sheath tumors can occur anywhere in the body. That mostly occur in the deep tissue of the arms, legs and trunk. They tend to cause pain and weakness in which they occur. They can also cause a growing lump or mass.

Surgery is the usual treatment for malignant peripheral nerve sheath tumors. Sometimes, the treatment may include radiation therapy and chemotherapy.

Symptoms

Malignant peripheral nerve sheath tumors often cause symptoms that worsen rapidly. The symptoms include:

  • Pain in the place where the tumor is growing.
  • Weakness when it comes to moving the part of the body that has the tumor.
  • A growing lump of tissue under the skin.

When to see a doctor

Make an appointment with your health care provider if you have ongoing symptoms that worry you. Malignant peripheral nerve sheath tumors are rare, so your provider may first check for more common causes of your symptoms.

Causes

It is not clear what the cause of the majority of the malignant peripheral nerve sheath tumors.

Experts know that these cancers start when a cell in the lining around a nerve has changes in their DNA. A cell's DNA contains the instructions that tell a cell what to do. The changes that tell the cells to make the cells more quickly. These cells continue living when healthy cells are killed as a part of their life cycle.

The cells form a mass called a tumor. The tumor can grow in and kill the healthy body tissue. In time, the cells can spread to other parts of the body.

Risk factors

The factors that increase the risk of malignant peripheral nerve sheath tumors include:

  • Radiation therapy for cancer. A malignant peripheral nerve sheath tumor can occur in the area being treated with radiation for 10 to 20 years after treatment.
  • They are not cancerous, tumors of the nerve. Malignant peripheral nerve sheath tumors can develop from nerve tumors that are not cancerous, such as neurofibroma.
  • A condition that runs in families. Malignant peripheral nerve sheath tumors occur more frequently in people with neurofibromatosis type 1. This condition increases the risk of tumors in the nerves.

Diagnosis

Tests and procedures used to diagnose malignant peripheral nerve sheath tumors include:

  • Neurological examination. A detailed examination of the nervous system, known as a neurological examination, helps a health care provider to gather clues to the diagnosis.
  • Imaging tests. Imaging tests create pictures of the body. The images can help providers see the size of the cancer and whether it has spread to other parts of the body. The tests may include magnetic resonance imaging, also called magnetic resonance imaging , magnetic resonance neurography. Other tests may include ct scan, also called computed tomography, and positron emission tomography, also called PET.
  • The removal of a sample of tissue for analysis.A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue can be removed through a needle placed through the skin and the cancer. Sometimes surgery is necessary to obtain a tissue sample. The sample is analyzed in a laboratory to see if it is cancer. Other special tests to give more details on the cancer cells. The health care team uses this information to make a treatment plan.

The removal of a sample of tissue for analysis. A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue can be removed through a needle placed through the skin and the cancer. Sometimes surgery is necessary to obtain a tissue sample.

The sample is analyzed in a laboratory to see if it is cancer. Other special tests to give more details on the cancer cells. The health care team uses this information to make a treatment plan.

Treatment

Treatment for malignant peripheral nerve sheath tumors often involves:

  • Surgery.The goal of surgery is to remove the tumor and some healthy tissue around it. When you can't do that, surgeons remove as much of the tumor as possible. Depending on where malignant peripheral nerve sheath tumor is and how big it is, the surgery can cause nerve damage. In the case of tumors that occur in the arms and legs, the surgical removal of the arm or leg may be necessary. Sometimes, the radiation before surgery can reduce the size of a tumor. That could make it more likely that all of the tumor is removed during surgery.
  • The radiation therapy.Radiation therapy uses high-energy rays to kill cancer cells. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body. Radiation may be used before surgery to shrink the tumor. This may make it more likely that all of the tumor is removed during surgery. After surgery, radiation therapy can be used to kill cancer cells that may remain.
  • Chemotherapy. Chemotherapy uses strong drugs to kill cancer cells. Chemotherapy may be an option when a malignant tumor of peripheral nerve sheath tumor has spread to other parts of the body. Chemotherapy can help control the symptoms and slow the growth of cancer.
  • Rehabilitation. After surgery, physical therapists, and occupational therapists can help you regain function, and the lost motion due to nerve damage or the removal of an arm or a leg.

Surgery. The goal of surgery is to remove the tumor and some healthy tissue around it. When you can't do that, surgeons remove as much of the tumor as possible.

Depending on where malignant peripheral nerve sheath tumor is and how big it is, the surgery can cause nerve damage. In the case of tumors that occur in the arms and legs, the surgical removal of the arm or leg may be necessary.

Sometimes, the radiation before surgery can reduce the size of a tumor. That could make it more likely that all of the tumor is removed during surgery.

The radiation therapy. Radiation therapy uses high-energy rays to kill cancer cells. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points on your body.

Radiation may be used before surgery to shrink the tumor. This may make it more likely that all of the tumor is removed during surgery. After surgery, radiation therapy can be used to kill cancer cells that may remain.

Preparing for your appointment

If you have symptoms that worry you, make an appointment with your doctor.

If you are diagnosed with a malignant tumor of peripheral nerve sheath tumor, the more likely it is to be referred to a doctor who specializes in:

  • Conditions that affect the nervous system, known as a neurologist.
  • The treatment of cancer, known as an oncologist.
  • Surgery of the bones, known as an orthopedist.
  • Surgery of the nerves, known as a neurosurgeon.

Because appointments can be brief, and because there is a lot to discuss, it is a good idea to be prepared. Here's some information to help you to be prepared and know what to expect.

What you can do

Ask a family member or friend to go with you. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.

Make a list of:

  • Their symptoms, and when they began.
  • All the drugs, vitamins, and other supplements that you take, including over-dose.
  • Questions to ask your health care provider.

Some questions to ask at your first appointment are:

  • What can be causing my symptoms or condition?
  • There are other possible causes?
  • What tests do I need?
  • What is the next step in the search of my diagnosis and decide on treatment?
  • There are restrictions that must be followed in the interim?

Questions to ask a specialist:

  • I have a malignant peripheral nerve sheath tumor?
  • What are the goals of treatment for me?
  • What treatment do you suggest?
  • What do I need to start treatment right away?
  • I have these other health problems. How can I best manage them together?
  • What are the possible side effects of treatment?
  • If the treatment does not work, what's next?
  • What is the outlook for my condition?

Be sure to ask any other questions you may have.

What to expect from your doctor

Your health care provider is likely to ask questions such as:

  • The symptoms changed over time?
  • Is there something to improve the symptoms?
  • Is there anything worse?
  • You have said that you have other medical conditions?
Symptoms and treatment of Malignant peripheral nerve sheath tumors