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Malignant Peripheral Nerve Sheath Tumors: Causes, Symptoms, Risk Factors, Diagnosis and Treatment

Jan 10, 2024

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Causes Of Malignant Peripheral Nerve Sheath Tumors

Symptoms Of Malignant Peripheral Nerve Sheath Tumors

Risk Factors Of Malignant Peripheral Nerve Sheath Tumors

Diagnosis Of Malignant Peripheral Nerve Sheath Tumors

Treatment  Of Malignant Peripheral Nerve Sheath Tumors

Malignant Peripheral Nerve Sheath Tumors Causes, Symptoms, Risk Factors, Diagnosis and Treatment

Malignant peripheral nerve sheath tumors are uncommon cancers that start in the nerve lining. The nerves that leave the body following the spinal cord are known as peripheral nerves, and these cancers can damage them. Malignant tumors of the sheath surrounding peripheral nerves were formerly referred to as neurofibrosarcomas.

Malignant peripheral nerve sheath tumors can appear anywhere in the body. They are mostly seen in the deep tissue of the limbs, legs, and trunk. Whenever they manifest, they typically cause pain and weakness. They might also cause a lump or tumor to enlarge.

The usual treatment for malignant peripheral nerve sheath tumors is surgery. Treatments that are occasionally employed include radiation therapy and chemotherapy.


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Causes Of Malignant Peripheral Nerve Sheath Tumors

The cause of most malignant peripheral nerve sheath tumors is unknown.

Scientists know that the first cause of malignant cancers is changes in DNA that take place in a nerve-lining cell. A cell's DNA contains instructions that tell it what to do. The changes tell the cells to proliferate quickly. Healthy cells survive when they naturally perish as a part of their life cycle.

A tumor is a mass that may develop from cells over time. Healthy body tissue can be invaded and destroyed by tumors. Over time, the cells may move to other parts of the body.

Also Read: Scrotal Masses: Causes, Symptoms, Risk Factors, Diagnosis, Treatment and Complications

Symptoms Of Malignant Peripheral Nerve Sheath Tumors

Malignant peripheral nerve sheath tumors may present with quickly worsening symptoms. Among the signs and symptoms are:

  • Pain where the tumor is growing.
  • A lack of strength when trying to move the affected body part.
  • A growing mass of tissue under the skin.

Also Read: Morton's Neuroma: Causes, Symptoms, Risk Factors, Diagnosis and Treatment


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Risk Factors Of Malignant Peripheral Nerve Sheath Tumors

The subsequent factors heighten the likelihood of malignant peripheral nerve sheath tumors:

  • Radiation therapy for cancer: A malignant peripheral nerve sheath tumor may appear in the radiation-treated region ten to twenty years after therapy.
  • Benign neural growths: Malignant peripheral nerve sheath tumors can develop from non-cancerous nerve tumors, such as neurofibromas, an inherited condition. Malignant peripheral nerve sheath tumors are more common in people with neurofibromatosis 1. Nerve tumors are a potential with this condition.

Also Read: Bladder Exstrophy: Causes, Symptoms, Risk Factors, Diagnosis, Treatment and Complications

Diagnosis Of Malignant Peripheral Nerve Sheath Tumors

The following diagnostic techniques and tests can be used to identify malignant peripheral nerve sheath tumors:

  • Evaluation of the nervous system: A neurological exam, which is a comprehensive assessment of the nervous system, helps a medical practitioner gather diagnostic data.
  • Imaging tests: Images of the human body are produced using imaging tests. Medical personnel may use the photos to help assess the cancer's spread and determine its exact location within the body. There are two alternative test types: magnetic resonance imaging (MRI) and magnetic resonance neurography. Computed tomography, or CT scan, and positron emission tomography, or PET scans, may be used as additional testing methods.
  • Removing a sample of tissue for examination: A biopsy is the procedure of removing a sample of tissue for examination in a lab. The tissue may be removed using a needle injected through the skin and into the malignancy. A tissue sample may occasionally need to be obtained by surgery. The sample is examined in a lab to check for the presence of malignancy. Further specialized testing yields further details about the cancer cells. The medical staff uses this information to create a treatment plan.

Also Read: Renal Artery Stenosis: Causes, Symptoms, Risk Factors, Diagnosis, Treatment and Complications

Treatment  Of Malignant Peripheral Nerve Sheath Tumors

Treatments for malignant peripheral nerve sheath tumors frequently include:

  • Surgery: The goal of surgery is to remove the tumor together with a portion of the surrounding healthy tissue. When that is not possible, surgeons remove as much of the tumor as they can.
    • The degree and position of a malignant peripheral nerve sheath tumor dictate whether or not surgery causes nerve injury. It could be required to surgically remove the affected limb or leg when tumors grow in the arms or legs.
    • Sometimes radiation causes cancer to shrink before surgery. That might make it more likely that after surgery the tumor will be totally removed.
  • Radiation therapy: Radiation therapy uses high-energy beams to kill cancer cells. The energy may have originated from protons, X-rays, or other sources. For radiation therapy, you are surrounded by a machine while lying on a table. The device directs radiation onto certain body parts. Before surgery, radiation therapy may be used to shrink a tumor. This could make it more likely that, after surgery, the cancer will be removed. If cancer cells remain after surgery, they can be eliminated using radiation therapy.
  • Chemotherapy: Chemotherapy uses strong drugs to attack cancer cells. Chemotherapy may be an option if a malignant peripheral nerve sheath tumor has spread to other body parts. Chemotherapy may help control symptoms and stop the spread of cancer.
  • Rehabilitation: If nerve damage or having an arm or limb amputated has left you without mobility or function after surgery, physical and occupational therapists can help you restore it.

Also Read: Pancreatic Neuroendocrine Tumours: Causes, Symptoms, Diagnosis and Treatment

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