Malignant peripheral nerve sheath tumor causes
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]
Overview
Malignant peripheral nerve sheath tumor may be caused by a mutation in the neurofibromatosis type I gene.[1][2]
Causes
- Malignant peripheral nerve sheath tumor may be caused by a mutation in the neurofibromatosis type I gene.[1][2]
- About half of the cases of malignant peripheral nerve sheath tumor (MPNST) occur along with NF1. 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.[2]
- The NF1 gene locus is on chromosome 17q11.2 and the gene product is neurofibromin, which acts as a tumor suppressor. Inactivation of the gene predisposes to tumor development.[1]
References
- ↑ 1.0 1.1 1.2 Albright, A (2008). Principles and practice of pediatric neurosurgery. New York: Thieme. ISBN 1588903958.
- ↑ 2.0 2.1 2.2 Ferrari, Andrea; Gianni Bisogno; Modesto Carli (2007). "Management Of Childhood Malignant Peripheral Nerve Sheath Tumor". Pediatric Drugs. 9 (4): 239–48. doi:10.2165/00148581-200709040-00005. PMID 17705563.
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