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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Carcinoid tumor


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Your Question

In 2010 my brother was diagnosed with a carcinoid tumor in the rectum. Does this genetically or otherwise pre-dispose me to a carcinoid tumor?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What causes carcinoid tumors?

The cause of carcinoid tumors is unknown. However, there are some risk factors (none of which are avoidable) that may increase a person's risk of developing a carcinoid tumor. Although risk factors can influence the development of cancer, most do not directly cause cancer. People with several risk factors may never develop cancer, while others with no known risk factors do. Possible risk factors for carcinoid tumors include:[1]
  • Family history of multiple endocrine neoplasia type 1 (MEN1). MEN1 is a hereditary condition that increases the risk of developing tumors in the endocrine glands and small intestine. It is estimated that approximately 10% of gastrointestinal (GI) carcinoid tumors are associated with MEN1.
  • Race and gender. GI carcinoid tumors are more common among black people than white people, and black men have a higher risk than black women. Among white people, men and women have the same risk. Race and gender are not a significant risk factor in lung carcinoid tumors.
  • Age. For GI carcinoid tumors, the average age at diagnosis is 55 to 65. For carcinoid tumors of the appendix, the average age at diagnosis is about 40. For lung carcinoid tumors, the average age at diagnosis is between 45 and 55. Children rarely develop carcinoid tumors.
  • Other stomach conditions. People with diseases that damage the stomach and reduce acid production have a greater risk of developing a stomach carcinoid tumor. In particular, people with pernicious anemia have a higher risk of a stomach carcinoid tumor.
Lung carcinoid tumors are not caused by or related to smoking.[1]
Last updated: 4/9/2012

Can an individual be predisposed to develop a carcinoid tumor?

Carcinoid tumors generally have been considered to be sporadic, except for a small proportion which occur as a part of multiple endocrine neoplasia (MEN) syndromes. Carcinoid tumors most notably may occur in MEN1, and occasionally in MEN2.[2] These conditions are caused by mutations in the MEN1 and RET genes, respectively, and most cases are considered to have an autosomal dominant pattern of inheritance.[3] When associated with these familial syndromes, carcinoids are more likely to develop in the foregut, especially in the thymus and lung.[2]

It has been reported that even when a carcinoid tumor does not appear to be associated with MEN, there does appear to be an increased risk for developing a carcinoid tumor among first-degree relatives; this suggests that some carcinoid tumors may be familial. However, little data are available on the percentage of individuals with a carcinoid tumor that have a family history of carcinoid tumors.[2]
Last updated: 4/10/2012

References