Familial gastrointestinal carcinoid tumours and associated cancers

Ann Oncol. 2009 May;20(5):950-4. doi: 10.1093/annonc/mdn706. Epub 2009 Jan 15.


Background: Carcinoid tumours are neuroendocrine neoplasms mainly located in the gastrointestinal tract and bronchopulmonary system. Our study's aim was to characterise the familial nature of gastrointestinal carcinoid tumours using the Swedish Family-Cancer Database, which includes >11.5 million individuals.

Methods: Familial relative risks (RRs) of carcinoid tumours were estimated for individuals with family history of invasive cancers. RRs of invasive cancers for the offspring of patients with carcinoid tumours were calculated as well. RRs were based on Poisson regression.

Results: The risk of carcinoid tumours was significantly increased among individuals with a parental history of carcinoid tumours (RR 4.33). An association was also found between carcinoid tumours in the offspring and parental invasive cancers of the brain, breast, liver, endocrine glands and urinary organs. In addition, parental invasive cancers of the kidney as well as squamous cell skin cancer associated with small intestinal carcinoids in the offspring. The risk of carcinoid tumours was elevated among those whose siblings were affected by colon and rectal cancers.

Conclusion: The results describe the familial aggregation of carcinoid tumours with other noncarcinoid malignancies. Carcinoid tumours are rare and translating these results into screening programmes needs more research; however, gene finding studies should be stimulated.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Carcinoid Tumor / epidemiology
  • Carcinoid Tumor / genetics*
  • Databases as Topic
  • Gastrointestinal Neoplasms / epidemiology
  • Gastrointestinal Neoplasms / genetics*
  • Genetic Predisposition to Disease
  • Humans
  • Pedigree
  • Poisson Distribution
  • Registries
  • Risk Assessment
  • Risk Factors
  • Sweden / epidemiology