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Genetic and Rare Diseases Information Center (GARD)

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Carcinoid tumor


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Overview

What is a carcinoid tumor?

What causes carcinoid tumors?

What treatments are available for carcinoid tumor?

What is a carcinoid tumor?

A carcinoid tumor is a rare, slow-growing cancer that usually starts in the lining of the digestive tract (appendix, stomach, small intestine, colon, rectum) or in the lungs but may occur elsewhere.[1][2] Because carcinoid tumors grow slowly and don't produce symptoms in the early stages, affected individuals may have the tumor for years before being diagnosed. In later stages the tumor sometimes produces hormones that can cause carcinoid syndrome. The syndrome causes flushing of the face and upper chest, diarrhea, and trouble breathing. Surgery is the main treatment for a carcinoid tumor. If it does not spread to other parts of the body, surgery can typically cure the cancer.[1]
Last updated: 4/9/2012

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:[3]
  • 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.[3]
Last updated: 4/9/2012

What treatments are available for carcinoid tumor?

Treatment of carcinoid tumor depends on the location, size, and spread of the tumor.  Typically, surgery is the first step and aims to remove as much of the tumor as possible.[4]  If any tumor remains following surgery, or if the tumor has spread to distant parts of the body (metastasized), medications such as octreotideeverolimus, or other chemotherapy drugs may be used to treat this condition.[4][5]
Last updated: 10/23/2014

References
  1. Carcinoid Tumors. MedlinePlus. 2009; http://www.nlm.nih.gov/medlineplus/carcinoidtumors.html. Accessed 10/4/2009.
  2. Carcinoid tumors. MayoClinic.com. 2008; http://mayoclinic.com/print/carcinoid-tumors/DS00834/DSECTION=all&METHOD=print. Accessed 10/4/2009.
  3. Carcinoid Tumor. Cancer.Net. April 2011; http://www.cancer.net/patient/Cancer+Types/Carcinoid+Tumor. Accessed 4/9/2012.
  4. National Comprehensive Cancer Network. Neuroendocrine Tumors. NCCN Clinical Practice Guidelines in Oncology. 2014; http://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Accessed 10/22/2014.
  5. Tebbi CK. Carcinoid Tumor Treatment & Management. Medscape. 2014; http://emedicine.medscape.com/article/986050-treatment. Accessed 10/23/2014.


See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.