Neurofibrosarcoma

Infobox_Disease
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DiseasesDB = 32055
ICD10 =
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ICDO = 9540/3
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MeshID = D018319

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, neurofibrosarcoma is a type of cancer that causes malignant tumors in areas surrounding peripheral nerves. Neurfibrosarcoma is an advanced stage of [neurofibroma] , or neurofibromatosis (NF). 5% 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.

Causes- Genetics have been the main culprit for the cause of neurofibrosarcoma, although researchers still do not know the exact cause of the disease. Soft tissue sarcomas have been linked within families, so it is hypothesized that neurofibrosarcoma may be genetic. Evidence to support this hypothesis includes the fact that there is a 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, but being that p53 is inactivated in neurofibrosarcoma patients, they are much more susceptible to developing tumors.

Symptoms- A swelling in the extremities (arms or legs) that is often painless. There also may be difficulty in the movement of the arms or legs, whichever extremity has the tumor. Soreness is also typical in many patients.

Diagnosis- The most conclusive test that can be done on a patient who may potentially have neurofibrosarcoma would be a biopsy of the tumor, which is just a cell sample that is taken from the tumor itself. MRIs, X-Rays, CAT scans, and bone scans may also be done to look for tumors and/or possible metastasis.

Treatment- The treatment for neurofibrosarcoma isn’t unlike the treatment of other cancers. Surgery is an option; the removal of the tumor along with surrounding tissue may be vital for the patient’s survival. Being that tumors are often located in the extremities, amputation is sometimes necessary for people suffering from neurofibrosarcoma. Amputation is often the last resort and will be considered if the tumor is vascularized, or has a blood supply, and has many nerves going through it and/ or around it. 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.
Chemotherapy drugs, along with a combination of those drugs, are also an option that may be considered when treating a patient with neurofibrosarcoma. Patients taking chemotherapy must be prepared for the side effects that come with any other chemotherapy treatment, such as; hair loss, lethargy, weakness, etc.

Prognosis How well the patient responds to treatments will vary from person to person. Age, health, and the patient tolerance to medications and therapies will have an immense effect on whether or not the patient fully recovers. Also, the extent of the disease i.e. metastasis and size/location of the tumor will too have an effect on the patient’s recovery

It is a rare tumor type, with a relatively poor prognosis.cite journal |author=Neville H, Corpron C, Blakely ML, Andrassy R |title=Pediatric neurofibrosarcoma |journal=J. Pediatr. Surg. |volume=38 |issue=3 |pages=343–6; discussion 343–6 |year=2003 |pmid=12632346 |doi=10.1053/jpsu.2003.50105]

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