Risks for neuroendocrine cancer

Last medical review:

Some things can affect your risk, or chance, of developing cancer. Certain behaviours, substances or conditions can increase or decrease the risk. Most cancers are the result of many risks. But sometimes cancer develops in people who don't have any risks.

Neuroendocrine cancer is rare. It can develop at any age, but most cases occur in middle-aged and older adults.

If you have a genetic condition that increases your risk for neuroendocrine cancer, you may need to visit your doctor more often. Talk to your doctor about your risk and if you need to have certain tests to check for neuroendocrine cancer.

There are a few things that could increase your risk for neuroendocrine cancer. None of these risks can be changed. Until we learn more about these risks, there are no specific ways you can lower your risk for neuroendocrine cancer.

The following can increase your risk for neuroendocrine cancer:

Genetic conditions

A genetic condition is a disease caused by a change (mutation) in one or more genes. Having certain genetic conditions increases the risk of developing neuroendocrine cancer.

Multiple endocrine neoplasia (MEN) is an inherited condition that is associated with tumours in more than one endocrine gland and an increased risk of developing endocrine system cancers.

There are different types of MEN:

  • MEN1 increases the risk of tumours in the parathyroid, pituitary and adrenal glands as well as the pancreas and ovaries.
  • MEN2A and MEN2B increase the risk of tumours in the thyroid (including medullary thyroid cancer), adrenal and parathyroid glands.
  • MEN4 increases the risk of tumours in the parathyroid and pituitary glands.

Von Hippel-Lindau (VHL) syndrome is an inherited condition that affects blood vessels in the eyes, brain, spinal cord, adrenal glands and other parts of the body, making them grow abnormally.

VHL syndrome increases the risk of kidney, adrenal gland, brain and pancreatic cancers.

Neurofibromatosis type 1 is an inherited condition that affects the nervous system. It affects the development and growth of neurons (nerve cells), causes tumours (neurofibromas) to grow on nerves and may produce other abnormalities in muscles, bones and skin. Sometimes cancerous tumours, called malignant peripheral nerve sheath tumours, may grow along the nerves.

Neurofibromatosis type 1 increases the risk of neuroendocrine cancer, soft tissue sarcoma, brain tumours, leukemia and neuroblastoma.

Tuberous sclerosis is an inherited condition that causes non-cancerous tumours to develop in the brain and spinal cord, skin, eyes, heart, lungs and kidneys. Tuberous sclerosis increases the risk of neuroendocrine cancer and kidney cancer.

Family history of cancer

Having a family history of any type of cancer, especially if the person affected by cancer is your first-degree relative, increases your risk for neuroendocrine cancer.

A family history of a specific type of cancer also increases the risk of developing neuroendocrine cancer in specific body parts. For example, a family history of lung cancer increases your risk of developing lung neuroendocrine cancer. A family history of colorectal or breast cancer is linked with developing neuroendocrine cancer in the small intestine.

Possible risks

The following have been linked with an increased risk of neuroendocrine cancer that starts in specific parts of the body, but more research is needed to know for sure that they are risks:

  • Smoking tobacco may increase the risk of neuroendocrine cancer that starts in the stomach, small intestine, pancreas or lungs (specifically atypical carcinoid tumours).
  • Chronic atrophic gastritis may increase the risk of neuroendocrine cancer that starts in the stomach.
  • Diabetes( also called diabetes mellitus) may increase the risk of neuroendocrine cancer that starts in the pancreas or stomach.

Understanding your cancer risk

To make the decisions that are right for you, ask your doctor questions about risks. Learn how cancer can be prevented and what you can do to reduce your risk.

Expert review and references

  • Canadian Cancer Society | Société canadienne du cancer
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  • National Comprehensive Cancer Network. NCCN Guidelines for Patients: Neuroendorine Tumours. 2022.
  • PDQ Adult Treatment Editorial Board. Gastrointestinal Neuroendocrine Tumors Treatment (PDQ®) – Health Professional Version . Bethesda, MD: National Cancer Institute; 2024. https://www.cancer.gov/.
  • PDQ Adult Treatment Editorial Board. Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®) – Health Professional Version. Bethesda, MD: National Cancer Institute; 2024. https://www.cancer.gov/.
  • Rossi RE, Massironi S. The increasing incidence of neuroendocrine neoplasms worldwide: current knowledge and open issues. Journal of Clinical Medicine . 2022: Jun 30;11(13):3794.
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