FaCD Online Syndrome Fact Sheet

Last updated: 10 Feb 2011

Name: Carcinoid, Familial Clustering of

Synonym: incl.: Carcinoid, Intestinal; incl. Familial Ileal Endocrine Carcinoma (FIEC).

Mode of Inheritance: multifact?/ AD?

OMIM number: 114900  

Tumor features

carcinoid of the lung
gastrointestinal carcinoid

Tumor features (possible)

brain tumor
breast cancer
colorectal cancer
esophageal cancer
hepatocellular cancer (hepatoma)
lung/bronchial cancer
prostate cancer
renal cell cancer
urinary bladder cancer


Anderson reported a father and daughter both diagnosed with carcinoid of the appendix (at age 42 and 15 years, respectively)[1]. Moertel and Dockerty[2] reported on a 2-generation family with (multiple) intestinal carcinoids. One of the affected women developed breast cancer at age 53 while her mother had done so at age 47. Other tumors in that family included nasopharyngeal fibrosarcoma (age 5) and lung cancer (age 42), possibly suggesting Li-Fraumeni-like syndrome (see that entry). Additional cases of familial intestinal carcinoids have been published [3-5, 8-10,14,15]. An unknown proportion of these and other cases of familial carcinoid might very well be caused by germline mutations in MEN1, the gene associated with Multiple Endocrine Neoplasia type 1. Cunningham et al[14] coined the name Familial Ileal Endocrine Carcinoma (FIEC) to refer to familial clustering of ileal carcinoid, which they suggest is an autosomal dominant and clinically distinct disorder.
Perkins et al.[6] studied the family history of patients with carcinoid tumors of the lung and demonstrated that the proportion of distant metastasis in these patients was significantly higher among those with a positive family history of cancer (in general). Babovic-Vuksanovic et al.[7] studied the occurrence of cancer in first-degree relatives of 245 gastrointestinal carcinoid patients. For first-degree relatives, only the risk for carcinoid was increased (P<0.0001, life-time risk 1.5%). In a recent study by Hiripi et al on gastrointestinal carcinoids, the risk to develop carcinoid tumours was increased for children of a parent with a carcinoid tumour(RR 4.3) or cancer of the brain, breast, liver, endocrine glands or urinary organs. Having a sibling affected with colorectal cancer increased the carcinoid risk as well[13].
Oliveira et al [11] reported pulmonary carcinoid in two affected sibs and in a mother and daughter. No other signs of MEN1 were present in these two families.
Hassan et al studied the association between risk to develop neuroendocrine tumors (NET), including carcinoids, and the family history of cancer[12]. Having first-degree relatives with cancer increased the risk to develop NETs of the small intestine, stomach, lung, and pancreas. Family history of esophageal cancer was associated with pancreatic NETs. Small intestinal NETs were associated with a family history of colorectal or prostate cancer. Pulmonary NETs were associated with a family history of lung cancer.


[1] Anderson RE. A familial instance of appendiceal carcinoid. Am J Surg 1966; 111(5):738-740.
[2] Moertel CG, Dockerty MB. Familial occurrence of metastasizing carcinoid tumors. Ann Intern Med 1973; 78(3):389-390.
[3] Wale RJ, Williams JA, Beeley AH, Hughes ES. Familial occurrence in carcinoid tumours. Aust N Z J Surg 1983; 53(4):325-328.
[4] Methfessel HD, Bettzieche H, Methfessel G. [Pregnancy and birth in appendix carcinoid tumor with familial disposition] Schwangerschaft und Geburt bei appendixkarzinoid mit familiarer disposition. Zentralbl Gynakol 1973; 95(7):234-238.
[5] Yeatman TJ, Sharp JV, Kimura AK. Can susceptibility to carcinoid tumors be inherited? Cancer 1989; 63(2):390-393.
[6] Perkins P, Lee JRJ, Kemp BL, Cox JD. Carcinoid tumors of the lung and family history of cancer. J Clin Epidemiol 50[6], 705-709. 1997.
[7] Babovic-Vuksanovic D, Constantinou CL, Rubin J, Rowland CM, Schaid DJ, Karnes PS. Familial occurrence of carcinoid tumors and association with other malignant neoplasms. Cancer Epidemiol Biomarkers Prev 1999; 8(8):715-719.
[8] Kinova S, Duris I, Kovacova E, Stvrtina S, Galbavy S, Makaiova I. Malignant carcinoid in two brothers. Bratislavské lekárske listy 2001; 102(5):231-4.
[9] Pal T, Liede A, Mitchell M, Calender A, Narod SA. Intestinal carcinoid tumours in a father and daughter. Canadian journal of gastroenterology 2001; 15(6):405-9.
[10] Katdare MV, Fichera A, Heimann TM. Familial rectal carcinoid: report of two first-degree relatives with rectal carcinoid and review of the literature. Techniques in coloproctology 2006; 10(2):143-6.
[11] Oliveira AM, Tazelaar HD, Wentzlaff KA, Kosugi NS, Hai N, Benson A, Miller DL, Yang P. Familial pulmonary carcinoid tumors. Cancer 2001; 91(11):2104-9.
[12] Hassan MM, Phan A, Li D, Dagohoy CG, Leary C, Yao JC. Family history of cancer and associated risk of developing neuroendocrine tumors: a case-control study. Cancer epidemiology, biomarkers & prevention 2008; 17(4):959-65.
[13] Hiripi E, Bermejo JL, Sundquist J, Hemminki K. Familial gastrointestinal carcinoid tumours and associated cancers. Ann Oncol. 2009 Jan 15. [Epub ahead of print]
[14] Cunningham JL, Díaz de Ståhl T, Sjöblom T, Westin G, Dumanski JP, Janson ET. Common pathogenetic mechanism involving human chromosome 18 in familial and sporadic ileal carcinoid tumors. Genes Chromosomes Cancer. 2011 Feb;50(2):82-94.
[15] Järhult J, Landerholm K, Falkmer S, Nordenskjöld M, Sundler F, Wierup N. First report on metastasizing small bowel carcinoids in first-degree relatives in three generations. Neuroendocrinology. 2010;91(4):318-23.