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Annals of Oncology Advance Access originally published online on January 15, 2009
Annals of Oncology 2009 20(5):950-954; doi:10.1093/annonc/mdn706
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© The Author 2009. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved. For permissions, please email: journals.permissions@oxfordjournals.org

epidemiology

Familial gastrointestinal carcinoid tumours and associated cancers

E. Hiripi1,*, J. Lorenzo Bermejo1, J. Sundquist2 and K. Hemminki1,2

1 Division of Molecular Genetic Epidemiology, German Cancer Research Center (DKFZ), Heidelberg, Germany
2 Karolinska Institute, Center for Family and Community Medicine, Huddinge, Sweden

* Correspondence to: Ms. E. Hiripi, Division of Molecular Genetic Epidemiology, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 580, Heidelberg 69120, Germany. Tel: +49-6221-421805; Fax: +49-6221-421810; E-mail: e.hiripi{at}dkfz-heidelberg.de

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.

Key words: carcinoid tumours, gastrointestinal tumours

Received for publication April 15, 2008. Revision received September 23, 2008. Accepted for publication October 10, 2008.


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