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Neurofibrosarcoma is a type of tumor combining characteristics of neurofibroma and fibrosarcoma.
Neurofibrosarcoma, also known as peripheral nerve sheath tumor, malignant schwannoma, malignant nerve sheath tumor, and neurogenic sarcoma, is a type of cancer that causes malignant tumors in areas surrounding peripheral nerves. Neurfibrosarcoma is an advanced stage of [neurofibroma] or neurofibromatosis (NF). Five percent of NF patients will develop neurofibrosarcoma at some point in their life. Patients who suffer from NF have up to a 100,000 fold increase in likelihood of developing neurofibrosarcoma when compared to the general population. It is often found in the arms and legs, but severe cases have been recorded as spreading to the lungs.
Soft tissue sarcomas have been linked within families, so it is hypothesized that neurofibrosarcoma may be genetic, although researchers still do not know the exact cause of the disease. Evidence supporting this hypothesis includes loss of heterozygosity on the 17p chromosome. The p53 (a tumor suppressor gene in the normal population) genome on 17p in neurofibrosarcoma patients is mutated, increasing the probability of cancer. The normal p53 gene will regulate cell growth and inhibit any uncontrollable cell growth in the healthy population; since p53 is inactivated in neurofibrosarcoma patients, they are much more susceptible to developing tumors.
Symptoms include
The most conclusive test for a patient with a potential neurofibrosarcoma is tumor biopsy, or taking a cell sample directly from the tumor itself. MRIs, X-rays (radiographs), CAT scans, and bone scans can aid in locating a tumors and/or possible metastasis.
Treatment for neurofibrosarcoma is similar to that of other cancers. Surgery is an option; the removal of the tumor along with surrounding tissue may be vital for the patient’s survival. For discrete, localized tumors, surgery is often followed by radiation therapy of the excised area to reduce the chance of recurrence. For patients suffering from neurofibrosarcomas in an extremity, if the tumor is vascularized (has its own blood supply) and has many nerves going through it and/or around it, amputation of the extremity may be necessary. Some surgeons argue that amputation should be the procedure of choice when possible, due to the increased chance of a better quality of life. Radiation will also be used in conjunction with surgery, especially if the limb was not amputated. Radiation is rarely used as a sole treatment. In some instances, the oncologist may choose Chemotherapy drugs when treating a patient with neurofibrosarcoma, usually in conjunction with surgery. Patients taking chemotherapy must be prepared for the side effects that come with any other chemotherapy treatment, such as; hair loss, lethargy, weakness, etc.
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