Malignant peripheral nerve sheath tumor


A malignant peripheral nerve sheath tumor is a type of cancer that affects the nerve linings that start from the spinal cord and enter other body parts. Earlier, these tumors were known by the name-neurofibrosarcomas.


Malignant peripheral nerve sheath tumors mostly occur in the deeper tissues of the legs, arms, and trunk. But they can affect any part of the body. The affected area becomes weak and pain develops. You may develop a mass or a lump that keeps on growing.


Usually, surgery helps treat a malignant peripheral nerve sheath tumor. However, in some cases, other treatments like chemotherapy or radiation therapy may also be helpful.


There is no clear reason for malignant peripheral nerve sheath tumors.


However, the cells that line the nerves develop certain mutations (errors) in genetic code. After that, the cells start diving quickly because of these mutations. This leads to the formation of more and more cells. The normal cells will die with time, while cancer cells remain.


In addition to this, several risk factors can increase your chance of developing a malignant peripheral nerve sheath tumor.


Risk factors

Previous radiation therapy for cancer. After treatment with radiation therapy, you may develop a malignant peripheral nerve sheath tumor. This can happen after a long time that is 10 to 20 years later.


Noncancerous nerve tumors. If you have a non-cancerous benign) nerve tumor-like neurofibroma, you can also develop a malignant peripheral nerve sheath tumor.


Inheritance. Moreover, some inheritance factors increase your chance of getting a malignant peripheral nerve sheath tumor. For instance, people suffering from neurofibromatosis 1 show a higher frequency of malignant peripheral nerve sheath tumors as well.




There are several signs and symptoms of malignant peripheral nerve sheath tumor. These include;

Pain at the site of the tumor

The affected part becomes quite weaker that is you cannot move it.

A lump of tissue under the skin that grows continuously.


Thus, if you show such kinds of symptoms, you need to make an appointment with your doctor. Furthermore, these tumors are very rare, so your healthcare professional may look for more-common reasons for your condition.



Your doctor may use several tests and procedures to detect your malignant peripheral nerve sheath tumor.


Neurological examination. Your doctor may order you for a neurological examination. This will help your doctor to get comprehensive details about your cancer. He/she will gather all the clues about your tumor detection.


Imaging tests. These tests give various details about the tumor. To understand your malignant peripheral nerve sheath tumor, your doctor may order various tests.

These tests also give an idea about the size and location of your tumor. For instance, the main imaging tests include magnetic resonance neurography, MRI, CT scan, and positron emission tomography (PET).


Magnetic resonance imaging (MRI): A technician will do an MRI when you lie still and flat on a plain surface. Then, this surface will take you inside an MRI machine that has a tube shape. To create a detailed, 3-D image inside your body, it uses radio waves and a magnetic field.


Computed tomography scan (CAT/CT scan): Similar to the MRI, your doctor may also perform a CT scan. It connects with a computer and takes pictures.


Positron emission tomography scan (PET scan): I this test, a technician will inject a small amount of radioactive sugar into your vein and place you inside a scanner. As the tumor cells use more sugar, they will brighten up in the scan.


Biopsy: In this method, using a needle or during surgery, your doctor will remove a piece of your tissue. To detect cancer, an expert views this sample under a microscope.

This will help confirm malignant peripheral nerve sheath tumors. All the information that your doctor gathers by these tests, helps your doctor to decide the best treatment option for you.




The main treatment options include:


The motive of surgery is to remove all the tumors. But, if the tumor has spread to other areas, it may not be possible to remove a malignant peripheral nerve sheath tumor. This is important when the tumor has spread to some vital organs.


However, other treatment options may be helpful if the surgery is not successful. This is important to kill all the remaining cancer cells with the help of other treatments, like radiation and chemotherapy.

This is because in certain cases, surgery may cause nerve damage and disability. At that time, limb amputation may become necessary. Furthermore, this is going to depend on the locations and size of your tumor.

Radiation therapy. 

To kill the cancer cells, your doctor may use radiation therapy that utilizes high-energy beams, like X-rays and protons. Your doctor may order radiation therapy to kill the remaining cancer cells. Thus, if it is not possible for your doctor to make surgery, he/she may employ radiation therapy.

Chemotherapy. This method uses powerful drugs or chemicals to kill the tumor cells of malignant peripheral nerve sheath tumors. In case the tumor has spread to other organs of the body, then your doctor may order chemotherapy.


Furthermore, in many cases, your doctor may use a combination of surgery, radiation, and chemotherapy to treat the malignant peripheral nerve sheath tumor in a better way.


Rehabilitation. After your doctor performs surgery, he/she may refer you to occupational therapists and physical therapists to recover. They will help you regain the mobility that was lost because of limb amputation.

Coping and support 


When you get diagnosed with malignant peripheral nerve sheath tumor, it could be frightening for you. The condition will be no different for your family.


However, you may learn to cope with the uncertainty and distress related to cancer with time.  Thus, you need to follow these instructions until then;


Call on for medical support:


Various things are going to help you. For instance, the knowledge and understanding of a medical social worker, or any other mental health professional is necessary. They will help you in understanding your cancer.


Furthermore, if your child or other family member suffers from cancer, you need to ask health care professionals for advice. They will provide you with options for medical health support. This will also provide you with emotional and social support.


In addition to this, you can check various online services that will provide you support to combat cancer.


  • Gain more knowledge about malignant peripheral nerve sheath tumor to make decisionsabout control and care: 


Ask your doctor about various treatment options related to malignant peripheral nerve sheath tumor. Little knowledge is dangerous. Therefore, more confidence in understanding and making decisions about treatment options will be there with you. So you should always learn more about the disease.


Ask the health care team for guidance if your child has cancer. Therefore, get more and more information for appropriately caring for the patient.


Be close to your friends and family:

A close and strong relationship with your family and friends is necessary. It will help you deal with malignant peripheral nerve sheath tumor.


You need practical support, moral support from your friends and relatives. Thus, someone should be there for you to look after the family. Emotional support from them is going to matter most. Thus, a healthy and happy person will ultimately efficiently fight the disease.


Prepare for your appointment


You are likely to start making an appointment with your primary care doctor if some signs and symptoms worry you. Ask for a referral to an experienced specialist if your doctor suspects malignant peripheral nerve sheath tumor.


A team of specialists typically can treat. For instance;

Tumor surgeons who have specialization in operating soft tissue cancers.

Doctors who have a specialization in treating cancers with systemic medications or chemotherapy.

Pathologists diagnose the specific type of cancer by analyzing a tissue.

Rehabilitation specialists who after surgery help in the recovery of a tumor.


What you should expect from your doctor:


You will face several questions from your doctor. Thus, you should be ready to answer these. So, give more time to your doctor to address them. Your doctor may ask;


What signs and symptoms concern you more?


Have your symptoms been occasional or continuous?


When did you start to notice the symptoms?

The severity of your symptoms?


Is there anything that improves your symptoms?


Is there anything that worsens your symptoms?


Do you have any family or personal history of cancer?