Risks and causes of neuroendocrine cancer

We don’t know what causes most neuroendocrine cancers. But there are some risks factors that can increase your risk of developing it. These include an inherited condition called MEN 1.

Anything that can increase your risk of getting a disease is called a risk factor.

Different cancers have different risk factors.­ Having one or more of these risk factors doesn't mean you will definitely get that cancer.

There are 2 key groups of neuroendocrine cancer:

  • neuroendocrine tumours (NETs Open a glossary item)
  • neuroendocrine carcinomas (NECs Open a glossary item)

Neuroendocrine cancer starts in different parts of your body. Where it starts is called the primary site.

The possible risks and causes of NETs and NECs are different. And the risks also depend on your primary site. 

Inherited conditions

Most neuroendocrine cancers are not inherited Open a glossary item. Doctors call these sporadic cancers. This means they don't know why you have developed the cancer. There isn't a known genetic (inherited) link. 

But there are some rare inherited conditions that can run in families. These can increase your risk of developing some types of NET. 

Multiple endocrine neoplasia 1 (MEN1)

MEN1 is a rare inherited condition. It can cause tumours to develop in different parts of the body. For example, the pancreas Open a glossary item, parathyroid gland Open a glossary item and pituitary gland Open a glossary item. The tumours can be benign Open a glossary item or cancerous. 

People with MEN1 have a higher risk of developing NETs in the pancreas. The risk increases as you get older. Research has shown that by the age of 80, up to 80 out of 100 people with MEN 1 (up to 80%) get a NET in the pancreas or duodenum Open a glossary item.

Sometimes people with MEN 1 develop lung or stomach NETs. But this is less common than developing pancreatic NETs.

Neurofibromatosis type 1

Neurofibromatosis type 1 (NF1) is caused by a change in the NF1 gene. The faulty gene is usually passed on from parents to their children. 

NF1 can increase your risk of developing some cancer types. This includes neuroendocrine tumours (NETs).

Von Hippel-Lindau (VHL)

VHL is a rare inherited condition caused by a change in the von Hippel-Lindau gene. It can affect different parts of the body. People with VHL have an increased risk of developing different types of tumours. These include pancreatic NETs.

Tuberous sclerosis (TSC)

This is a rare inherited condition. It causes tumours to develop in different parts of the body. Most tumours are benign Open a glossary item

Researchers think that people with tuberous sclerosis may have a higher risk of developing a pancreatic NET But this is rare.

Other risk factors

Other risk factors include:

Age

You can get neuroendocrine cancer at any age. It is most common in people aged 55 and older.

Smoking

Smoking is the biggest preventable cause of cancer. Smoking increases your risk of developing lung neuroendocrine carcinoma (NEC).  

Even light or occasional smoking increases the risk of cancer. But your risk increases more the longer you smoke and the more you smoke. If you are a smoker, the best thing you can do for your health is quit.

Drinking alcohol

There are mixed results from the research looking at alcohol and neuroendocrine cancer risk. Some studies have shown that drinking heavily may increase the risk of pancreatic NETS.

The UK government guidelines advise both men and women to drink no more than 14 units of alcohol a week.

Diabetes

Diabetes is a disease that causes the blood sugar level to increase. There is some evidence that people with diabetes have an increased risk of developing a pancreatic NET.

Diabetes is a common disease. Most people with diabetes will not develop a NET of the pancreas.

Family history of cancer

Researchers have found that your risk of developing some types of neuroendocrine cancer might be higher if you have a close relative who has had cancer. A close relative is a parent or sibling.

Long term stomach inflammation

Some stomach NETs are linked to a condition called atrophic gastritis. This is a long term condition that causes inflammation of the stomach. Research has shown that there is a link between atrophic gastritis and type 1 stomach NET.

Are there other causes?

Stories about possible cancer causes are often in the media and it can be hard to know what’s true and what’s not. You may have heard of something that isn’t included here. This is because we only include a risk factor in this information if it is supported by good quality evidence.

  • Hereditary Syndromes Associated with Pancreatic and Lung Neuroendocrine Tumors
    N Papadopoulou-Marketou and others
    Cancers,  2024. Volume 16, Issue 11, Page 2075  

  • Update on the clinical management of multiple endocrine neoplasia type 1
    C Pieterman and others
    Clinical Endocrinology,  2022. Volume 97, Issue 4, Pages 409-423

  • Initiating Pancreatic Neuroendocrine Tumor (pNET) Screening in Young MEN1 Patients: Results From the DutchMEN Study Group
    M J Klein Haneveldand others
    Journal of Clinical Endocrinology Metabolism, 2021. Volume 106, Issue 12, pages 3515-3525

  • Comparison of Cancer Prevalence in Patients With Neurofibromatosis Type 1 at an Academic Cancer Center vs in the General Population From 1985 to 2020
    J P Landry and others
    JAMA Network Open, 2021. Volume 4, Issue 3, Page e210945

  • Incidence and survival of neuroendocrine neoplasia in England 1995−2018: A retrospective, population based study
    B White and others
    Lancet Regional Health Europe, 2022. Volume 23, pages 1 - 11

  • The information on this page is based on literature searches and specialist checking. We used many references and there are too many to list here. Please contact patientinformation@cancer.org.uk if you would like to see the full list of references we used for this information.

Last reviewed: 
01 Apr 2025
Next review due: 
01 Apr 2028

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