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Neurofibrosarcoma - Causes, Symptoms, Diagnosis, and Management

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Neurofibrosarcoma, or malignant peripheral nerve sheath tumor, is a rare sarcoma that originates in the cells of the nerve sheath. Read the article to know more.

Written by

Dr. Shikha

Medically reviewed by

Dr. Abhishek Juneja

Published At September 23, 2022
Reviewed AtMarch 2, 2023

What Is Neurofibrosarcoma?

Neurofibrosarcomas, or peripheral nerve sheath tumors, are malignant tumors that develop in the soft tissue that surrounds the peripheral nerves, which receive messages from the brain and induce voluntary movement. These tumors are most commonly discovered in the arms and legs, and they have the potential to spread far along with nerve tissue. Sarcomas are uncommon malignancies, and malignant peripheral nerve sheath tumor (MPNST) is a rare kind of sarcoma that accounts for just five to ten percent of all sarcoma occurrences.

MPNST is classified as an aggressive tumor because it has a 65% likelihood of growing back after surgery and a 40% chance of spreading to other regions of the body or metastasizing. It most commonly metastasizes to the lungs. Young adults and middle-aged individuals are the most commonly affected by neurofibrosarcoma. Malignant peripheral nerve sheath tumor is more common in individuals who have neurofibromatosis type 1 (NF1), a hereditary condition.

What Causes Neurofibrosarcoma?

Although the specific origin of neurofibrosarcoma is unknown, research has suggested that genes may play a role in the development of all soft tissue sarcomas. The cause of the majority of neurofibrosarcomas or malignant peripheral nerve sheath tumors is unknown. These malignancies start when a cell in the protective coating around a neuron develops a DNA (deoxyribonucleic acid) defect or a mutation, according to doctors. The mutation directs the cell to proliferate quickly and live while other cells would typically die. The collecting cells form a tumor, which can move to other parts of the body or invade neighboring tissue.

The following factors raise the risk of neurofibrosarcoma or malignant peripheral nerve sheath tumors:

  • Nerve tumors that are not malignant such as neurofibroma can evolve into malignant peripheral nerve sheath tumors.

  • A neurofibrosarcoma or malignant peripheral nerve sheath tumor may occur after ten to 20 years in the area treated with radiation previously while managing cancer.

  • A hereditary disorder that raises the chance of nerve tumors in neurofibromatosis 1 or NF 1 whose patients are more likely to develop this malignancy.

What Are the Symptoms Observed in Neurofibrosarcoma?

Neurofibrosarcoma frequently does not exhibit any symptoms until it has been present for a long time. A painless swelling or apparent lump, soreness or limping due to compressed muscles or nerves, or other problems while using the arms, legs, feet, or hands are all common symptoms. Neurofibrosarcoma or malignant peripheral nerve sheath tumors can go undetected for a long time because they involve elastic tissues which can easily move. Nerve loss can also be observed.

How Is Neurofibrosarcoma Diagnosed?

A doctor may request the following tests to identify a malignant peripheral nerve sheath tumor or neurofibrosarcoma in addition to a comprehensive physical examination.

  • Biopsy: The doctor will perform a biopsy to determine if the tumor is a malignant peripheral nerve sheath tumor by extracting a tiny sample from the tumor using a needle. A pathologist will examine cells from the specimen under a microscope and perform additional tests to determine what type of tumor it is.

  • Imaging: If the patient has malignant peripheral nerve sheath tumor or neurofibrosarcoma symptoms, the doctor will utilize scans such as magnetic resonance imaging (MRI), computerized tomography (CT), and positron emission tomography (PET) to determine the location and size of the tumor. They will look for symptoms that indicate that the tumor has progressed to other places of the body as well.

  • Blood Test: A blood test and complete blood count can also be conducted by the doctor.

The tumor is given a stage after it has been diagnosed as neurofibrosarcoma, which is commonly stages I, II, III, or IV. This shows how far the tumor has spread from its initial point. The stage aids clinicians in determining the best course of treatment and predicting how the illness will respond to therapy. Neurofibrosarcoma can be localized, which means it has not moved beyond the nerve tissue where it started, or metastatic, which means it has gone beyond the nerve tissue where it began. Neurofibrosarcoma, on the other hand, is usually localized.

What Is the Management for Neurofibrosarcoma?

Neurofibrosarcoma or malignant peripheral nerve sheath tumors are commonly treated using the following treatment modalities:

  • Surgery: The goal while performing the surgery is to remove the entire tumor as well as a narrow margin of healthy tissue surrounding it. When this is not an option, doctors will remove as much of the tumor as feasible. Surgery can lead to complications causing damage to the nerves and disability depending on the location and size of the malignant peripheral nerve sheath tumor. Amputation of limbs may be required in the event of malignancies in the arms and legs. To decrease a tumor, the doctor may consider radiation before surgery in some situations. This may increase the chances of the tumor being entirely eliminated.

  • Radiation: Radiation therapy is a type of treatment that involves the use of intense beams of radiation energy such as X-rays and protons to kill cancer cells. Before surgery, radiation may be used to reduce a tumor and increase the chances of a successful surgery. Radiation therapy can also be performed after surgery to eradicate any cancer cells that remain.

  • Chemotherapy: Chemotherapy is a treatment that kills cancer cells by using chemicals. If the neurofibrosarcoma has metastasized to other parts of the body, the doctor may suggest chemotherapy to help the patient manage their symptoms and postpone the cancer progression.

  • Rehabilitation: Physical and occupational therapists can assist the patient in regaining mobility and function, which is lost due to amputation or nerve damage that can happen after surgery.

Conclusion:

Prognosis is a prediction of how a condition will affect you in the long run. Each person is unique, and the prognosis is based on a variety of characteristics, including the location of the tumor in the body, if the disease has spread to other parts of the body, and what percentage of the tumor was removed during surgery. It is critical to speak with a doctor if the patient wants to learn more about the prognosis. It is extremely difficult to deal with a rare type of cancer, and knowing more about the sarcoma can assist the patient in making decisions and coping with situations.

Frequently Asked Questions

1.

What Is Neurofibrosarcoma?

Neurofibrosarcomas, or peripheral nerve sheath tumors, are malignant tumors that develop in the soft tissue surrounding the peripheral nerves, which receive messages from the brain and induce voluntary movement. These tumors are most commonly discovered in the arms and legs.

2.

What Is the Recurrence Rate for Neurofibrosarcoma?

Neurofibrosarcoma is classified as an aggressive tumor because it has a 65% likelihood of growing back after surgery and a 40% chance of spreading to other body regions or metastasizing. It most commonly metastasizes to the lungs. Young adults and middle-aged individuals are the most frequently affected by Neurofibrosarcoma.

3.

What Is the Prognosis of Neurofibrosarcoma?

Each person is unique, and the prognosis is based on various characteristics, including the location of the tumor in the body, if the disease has spread to other parts of the body, and what percentage of the cancer was removed during surgery.

4.

What Causes Neurofibrosarcoma?

The causes of most neurofibrosarcomas or malignant peripheral nerve sheath tumors are unknown. According to doctors, these malignancies start when a cell in the protective coating around a neuron develops a DNA (deoxyribonucleic acid) defect or a mutation.

5.

What Triggers Sarcoma?

A neurofibrosarcoma or malignant peripheral nerve sheath tumor may occur after ten to 20 years in the area treated with radiation previously while managing cancer. A hereditary disorder that raises the chance of nerve tumors in neurofibromatosis, one or NF 1 patients are more likely to develop this malignancy.

6.

Does Sarcoma Run in Families?

Sarcomas are uncommon malignancies, and malignant peripheral nerve sheath tumor (MPNST) is a rare kind of sarcoma that accounts for just five to ten percent of all sarcoma occurrences. Malignant peripheral nerve sheath tumor is more common in individuals with neurofibromatosis type 1 (NF1), a hereditary condition.

7.

What Are the Symptoms Observed in Neurofibrosarcoma?

Neurofibrosarcoma frequently does not exhibit any symptoms until it has been present for a long time. Common symptoms include a painless swelling or apparent lump, soreness or limping due to compressed muscles or nerves, or other problems while using the arms, legs, feet, or hands.

8.

How Is Neurofibrosarcoma Diagnosed?

The doctor will perform a biopsy to determine if the tumor is a malignant peripheral nerve sheath tumor by extracting a tiny sample from the tumor using a needle. Then, a pathologist will examine cells from the specimen under a microscope and perform additional tests to determine what type of tumor it is.

9.

Can Sarcoma Be Entirely Cured by Surgery?

Surgery can lead to complications causing damage to the nerves and disability depending on the location and size of the malignant peripheral nerve sheath tumor. In addition, amputation of limbs may be required in the event of malignancies in the arms and legs. However, this may increase the chances of the cancer being eliminated.

10.

How to Manage Neurofibrosarcoma?

Neurofibrosarcoma or malignant peripheral nerve sheath tumors are commonly treated using the following treatment modalities:
- Surgery.
- Radiation.
- Chemotherapy.
- Rehabilitation.
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Dr. Abhishek Juneja
Dr. Abhishek Juneja

Neurology

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