Neurofibrosarcoma is a rare type of soft tissue sarcoma that develops around peripheral nerves. It may cause pain, soreness, and a noticeable lump under the skin. Treatment options include chemotherapy, radiation therapy, and surgery. Early diagnosis and treatment can improve the prognosis.
A neurofibrosarcoma is a rare type of tumor. It is also sometimes called a peripheral nerve sheath tumor. This type of growth only develops in the areas around the peripheral nerves, which are the parts of the nervous system that don’t include the brain and spinal cord. Examples include the nerves that run along the arms and legs.
Neurofibrosarcomas are classified as soft tissue sarcomas, which are cancers that start in the body’s tissues. Unlike many types of cancer, a neurofibrosarcoma typically doesn’t spread or metastasize. When it spreads, it commonly attacks the lungs.
A patient can live with a neurofibrosarcoma for a long time before experiencing any symptoms. This is because it exists in soft tissue that can be pushed aside to make room for the growing tumor. Some common symptoms may include soreness and pain, which may be accompanied by a noticeable lump under the skin. Patients may also limp and have trouble using their limbs normally.
It is not known exactly what causes a neurofibrosarcoma to develop. They can occur due to genetic mutations. A person with this type of cancer may also have a higher risk of developing other types of cancer. Patients with Recklinghausen’s disease, also known as neurofibromatosis, are at increased risk for neurofibrosarcomas. Neurofibromatosis is a genetic disease associated with a specific mutated gene and the development of other types of benign or harmless tumors.
A doctor who suspects a patient may have soft tissue sarcoma often orders X-rays and a magnetic resonance imaging (MRI) procedure. He may also require a computed tomography (CT) scan. These diagnostic tests will allow your doctor to examine and evaluate potential growth. Blood tests may also be needed.
To confirm the diagnosis, a biopsy is often needed. This is a surgical procedure in which a small sample of neurofibrosarcoma tissue is extracted. It can then be studied in the laboratory.
The treatment recommended for this type of cancer often depends on how aggressive the tumor is and how far it has spread, as well as the patient’s overall health. Chemotherapy or radiation therapy may be recommended to shrink the tumors and destroy the cancer. These therapies can be used alone or together.
In some cases, your doctor may recommend surgery. Surgical removal of the tumor may require partial or complete removal of the affected limb. Sometimes, the limb can be saved through a process called limb salvage surgery or limb preserving surgery. Patients who undergo this procedure, in which as much of the limb as possible is preserved, may need a full year of physical therapy to regain full use. Other times, an amputation, or removal of the limb, is necessary to preserve the patient’s life.
The overall prognosis, or expected outcome, for a neurofibrosarcoma can vary widely from patient to patient. It will depend on the location and size of the growth, the overall health of the patient, and the aggressiveness of the cancer. Patients who receive early diagnosis and early treatment have a better chance of survival.
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