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Pancreatic Neuroendocrine Tumor Risk Factors

A risk factor is anything that increases your chance of getting a disease such as cancer. Different cancers have different risk factors. Some risk factors, like smoking, can be changed. Others, like a person’s age or family history, can’t be changed.

Having a risk factor, or even many risk factors, does not mean that you will get the disease. And some people who get the disease may have few or no known risk factors.

Several factors can affect a person’s chance of getting a pancreatic neuroendocrine tumor (pNET).

Pancreatic neuroendocrine tumor (pNET) risk factors that can be changed

Smoking

Smoking comes with health risks, including an increased risk for pNETs. Most research shows that heavy smoking increases risk, but some studies show that any history of smoking could put you at risk.

Alcohol

Some studies have shown a link between heavy alcohol use and pNETs. This link appears to be mostly related to functioning pNETs rather than non-functioning pNETs. Heavy alcohol use can also lead to conditions such as chronic pancreatitis, which may increase pNET risk.

Pancreatic neuroendocrine tumor (pNET) risk factors that can’t be changed

Family history

PNETs seem to run in some families. In some, the high risk is caused by an inherited syndrome (explained below). In other families, the gene causing the increased risk is not known. If family history is a risk factor, it usually involves a first-degree relative (parent, sibling, child), a family history of pNET, or a family history of any cancer.

Inherited genetic syndromes

Inherited gene changes (mutations) can be passed from parent to child. Sometimes these changes result in syndromes that increase risks for other cancers (or other health problems).

Pancreatic neuroendocrine tumors and cancers can also be caused by genetic syndromes, such as:

  • Neurofibromatosis, type 1, which is caused by mutations in the NF1 gene. This syndrome leads to an increased risk of many tumors, including somatostatinomas.
  • Multiple endocrine neoplasia, type I (MEN1), caused by mutations in the MEN1 gene. This syndrome leads to an increased risk of tumors of the parathyroid gland, the pituitary gland, and the islet cells of the pancreas.
  • Von Hippel-Lindau (VHL) syndrome, which is caused by mutations in the VHL gene. This syndrome leads to an increased risk of many tumors, including pancreatic NETs.

Changes in the genes that cause some of these syndromes can be found by genetic testing. For more information on genetic testing, see Can Pancreatic Neuroendocrine Tumor Be Found Early?

Diabetes

PNETs are more common in people with diabetes. The reason for this is not known. Most of the risk is found in people with type 2 diabetes. This type of diabetes most often starts in adulthood and is often related to excess weight or obesity. It’s not clear if people with type 1 (juvenile) diabetes have a higher risk.

Chronic pancreatitis

Chronic pancreatitis, a long-term inflammation of the pancreas, is linked with an increased risk of pancreatic NETs. If chronic pancreatitis is caused by heavy alcohol use, then stopping alcohol use may help decrease the risk of pancreatic NETs.

Factors with an unclear effect on pancreatic neuroendocrine tumor (pNET) risk

Excess weight or obesity

Excess weight or obesity could be a risk factor for pNET. Studies so far are inconclusive.

Coffee

Some older studies have suggested that drinking coffee might increase the risk of pNET, but more recent studies have not confirmed this.

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Developed by the American Cancer Society medical and editorial content team with medical review and contribution by the American Society of Clinical Oncology (ASCO).

American Society of Clinical Oncology. Von Hippel-Lindau Syndrome. Accessed at cancer.net. Content is no longer available. 

Ben Q, Zhong J, Fei J, et al. Risk Factors for Sporadic Pancreatic Neuroendocrine Tumors: A Case-Control Study. Scientific Reports. 2016;6:36073. doi:10.1038/srep36073.

Haugvik SP, Hedenstr?m P, Kors?th E, et al. Diabetes, smoking, alcohol use, and family history of cancer as risk factors for pancreatic neuroendocrine tumors: a systematic review and meta-analysis. Neuroendocrinology. 2015;101(2):133-42. doi: 10.1159/000375164. Epub 2015 Jan 22.

Leoncini E, Carioli G, La Vecchia C, Boccia S, Rindi G. Risk factors for neuroendocrine neoplasms: a systematic review and meta-analysis. Ann Oncol. 2016 Jan;27(1):68-81. doi: 10.1093/annonc/mdv505. Epub 2015 Oct 20.

National Cancer Institute. Physician Data Query (PDQ). Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment – Patient Version. 2018. Accessed at https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq on October 7, 2018.

Valente R, Hayes AJ, Haugvik SP, et al. Risk and protective factors for the occurrence of sporadic pancreatic endocrine neoplasms. Endocr Relat Cancer. 2017 Aug;24(8):405-414. doi: 10.1530/ERC-17-0040. Epub 2017 May 31.

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 October 9, 2018.

 

Last Revised: March 29, 2025

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