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Malignant peripheral nerve sheath tumor

A malignant peripheral nerve sheath tumor is a form of cancer of the connective tissue surrounding nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8-13%. MPNST with rhabdomyoblastomatous component are called malignant triton tumors. The first-line treatment is surgical resection with wide margins. Chemotherapy and often radiotherapy are done as adjuvant and/or neoadjuvant treatment. Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the soft tissue that surrounds nerves. They are a type of sarcoma. Most malignant peripheral nerve sheath tumors arise from the nerve plexuses that distribute nerves into the limbsthe brachial and lumbar plexusesor from nerves as they arise from the trunk. 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 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. A malignant peripheral nerve sheath tumor is rare, but is one of the most common frequent soft tissue sarcoma in the pediatrics population. About half of these cases also happen to occur along with NF1, which is a genetic mutation on the 17th chromosome which causes tumors along the nervous system. The lifetime risk of having both of these conditions is at 8–13% while those with only MPNST have a 0.001% in the general population. NF1 and MPNST are categorized as autosomal dominant disorders. This means when one receives an abnormal gene from one of their parents, they will ultimately have that disorder. That person has a 50/50 chance of passing on that gene to their offspring. The pedigree to the right describes this genetic pattern. The most conclusive test for a patient with a potential neurofibrosarcoma is a tumor biopsy. MRIs, X-rays, CT scans, and bone scans can aid in locating a tumor 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 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. Otherwise, surgeons may opt for a limb-saving treatment, by removing less of the surrounding tissue or part of the bone, which is replaced by a metal rod or grafts. 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. Patient response to treatment will vary based on age, health, and the tolerance to medications and therapies. Video Empire produces videos read aloud. Use the information in this video at your own risk. We cannot always guarantee accuracy. This video uses material from https://en.wikipedia.org/wiki/Maligna..., licensed with CC Attribution-ShareAlike 3.0. This video is licensed with CC Attribution-Share-Alike 3.0 https://creativecommons.org/licenses/... In order to adapt this content it is required to comply with the license terms. Image licensing information is available via: https://en.wikipedia.org/wiki/Maligna...