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Pancreatic Neuroendocrine Tumor (NET)
Scientists don¡¯t know exactly what causes most pancreatic neuroendocrine tumors (NETs), but they have found several risk factors that can make a person more likely to get this disease. Some of these risk factors affect the DNA of cells in the neuroendocrine system in the pancreas, which can result in abnormal cell growth and may cause cancers to form.
DNA is the chemical in our cells that carries our genes, which control how our cells function. We look like our parents because they are the source of our DNA. But DNA affects more than just how we look.
Some genes control when our cells grow, divide into new cells, and die:
Cancers can be caused by DNA changes (mutations) that turn on oncogenes or turn off tumor suppressor genes.
Although 90% of PNETs are sporadic (random), some people inherit gene changes from their parents that raise their risk of pancreatic NET. Sometimes these gene changes are part of syndromes that include increased risks of other health problems as well.
Syndromes related to changes in three tumor suppressor genes are responsible for many inherited cases of pancreatic NETs:
The treatment for a pancreatic NET that's caused by a genetic syndrome might be slightly different compared to treatment for a pancreatic NET in someone without a gene mutation.
Most gene mutations related to neuroendocrine tumors of the pancreas are caused by random changes. These random mutations are called acquired if they occur after a person is born, rather than having been inherited. These acquired gene mutations sometimes result from exposure to cancer-causing chemicals (like those found in tobacco smoke). But often what causes these changes is not known.
The ÃÛÌÒ´«Ã½ Cancer Society medical and editorial content team
Our team is made up of doctors and oncology certified nurses with deep knowledge of cancer care as well as editors and translators with extensive experience in medical writing.
Benafif S and Eeles R. Diagnosis and Management of Hereditary Carcinoids. Recent Results Cancer Res. 2016; 205:149-68. doi: 10.1007/978-3-319-29998-3_9.
Chai SM, Brown IS, Kumarasinghe MP. Gastroenteropancreatic neuroendocrine neoplasms: selected pathology review and molecular updates. Histopathology. 2018 Jan;72(1):153-167. doi: 10.1111/his.13367.
Chen M, Van Ness M, Guo Y, Gregg J. Molecular pathology of pancreatic neuroendocrine tumors. Journal of Gastrointestinal Oncology. 2012;3(3):182-188. doi:10.3978/j.issn.2078-6891.2012.018.
Friedman JM. Neurofibromatosis 1. 1998 Oct 2 [Updated 2018 May 17]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews? [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1109/
Lee J, Allendorf J, Chabot J. Surgical resection of sporadic pancreatic neuroendocrine tumors. UpToDate website. https://www.uptodate.com/contents/surgical-resection-of-sporadic-pancreatic-neuroendocrine-tumors. Updated January 09, 2018. Accessed October 3, 2018.
van Leeuwaarde RS, Ahmad S, Links TP, et al. Von Hippel-Lindau Syndrome. 2000 May 17 [Updated 2018 Sep 6]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews? [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1463/
Vinik A, Perry RR, Hughes MS, et al. Multiple Endocrine Neoplasia Type 1. [Updated 2017 Oct 7]. In: De Groot LJ, Chrousos G, Dungan K, et al., editors. Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK481897/. Accessed 10.02.18.
Yao JC, Evans DB. Chapter 85: Pancreatic neuroendocrine tumors. In: DeVita VT, Lawrence TS, Rosenberg SA, eds. DeVita, Hellman, and Rosenberg¡¯s Cancer: Principles and Practice of Oncology. 10th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2015.
Last Revised: October 30, 2018
ÃÛÌÒ´«Ã½ Cancer Society medical information is copyrighted material. For reprint requests, please see our Content Usage Policy.
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