Malignant peripheral nerve sheath tumor pathophysiology
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Malignant peripheral nerve sheath tumor Microchapters |
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Differentiating Malignant Peripheral Nerve Sheath Tumor from other Diseases |
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Diagnosis |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
Causes
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.
Genetics
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.[1] 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.