Extracolonic cancer risk in Dutch patients with APC (adenomatous polyposis coli)-associated polyposis

Autor: Wouter H. de Vos tot Nederveen Cappel, Zeinab Ghorbanoghli, Hans Morreau, Evelien Dekker, Jan J. Koornstra, Jan-Werner Poley, James C. H. Hardwick, Hans F. A. Vasen, Alexandra M. J. Langers, Ben J.M. Witteman, Fokko M. Nagengast, Barbara A. J. Bastiaansen, Silvia Sanduleanu
Přispěvatelé: Guided Treatment in Optimal Selected Cancer Patients (GUTS), CCA - Cancer Treatment and Quality of Life, Gastroenterology and Hepatology, Amsterdam Gastroenterology Endocrinology Metabolism, APH - Quality of Care, Gastroenterology & Hepatology, RS: GROW - R3 - Innovative Cancer Diagnostics & Therapy, MUMC+: MA Maag Darm Lever (9)
Jazyk: angličtina
Rok vydání: 2018
Předmět:
Oncology
Adult
Male
medicine.medical_specialty
Genes
APC

CARCINOMA
Colorectal cancer
Adenomatous polyposis coli
DUODENAL ADENOMATOSIS
NETHERLANDS
Disease
Familial adenomatous polyposis
03 medical and health sciences
0302 clinical medicine
All institutes and research themes of the Radboud University Medical Center
SDG 3 - Good Health and Well-being
Risk Factors
FAMILIAL ADENOMATOUS POLYPOSIS
Internal medicine
Cause of Death
Genetics
medicine
Tumours of the digestive tract Radboud Institute for Molecular Life Sciences [Radboudumc 14]
MANAGEMENT
Humans
Genetic Predisposition to Disease
Thyroid cancer
Genetics (clinical)
Cause of death
biology
business.industry
Cancer
medicine.disease
GENE
PREVALENCE
Adenomatous Polyposis Coli
030220 oncology & carcinogenesis
Mutation
biology.protein
REGISTRATION
030211 gastroenterology & hepatology
Female
Duodenal cancer
business
Colorectal Neoplasms
Zdroj: Journal of Medical Genetics, 55, 11-14
Journal of Medical Genetics, 55, 1, pp. 11-14
JOURNAL OF MEDICAL GENETICS, 55(1), 11-14. BMJ PUBLISHING GROUP
Journal of Medical Genetics, 55(1), 11-14
Journal of medical genetics, 55(1), 11-14. BMJ Publishing Group
Journal of Medical Genetics, 55(1), 11-14. BMJ Publishing Group
ISSN: 0022-2593
DOI: 10.1136/jmedgenet-2017-104545
Popis: BackgroundScreening of patients with familial adenomatous polyposis (FAP) have led to a substantial reduction in mortality due to colorectal cancer (CRC). Recent guidelines suggest that surveillance of non-intestinal malignancies should also be considered in those patients. However, the value of these surveillance programmes is unknown. The aims of this study were (1) to assess the occurrence of extracolonic malignancies in a large series of adenomatous polyposis coli (APC) mutation carriers and (2) to evaluate the causes of death.MethodsAll APC mutation carriers were selected from the Dutch polyposis registry. Data on causes of death were collected. Pathology reports were retrieved from the Dutch Pathology Registry.ResultsA total of 85 extracolonic malignancies were diagnosed in 74 of 582 APC mutation carriers. Duodenal and skin cancers were the most prevalent cancers. Thyroid cancer was observed in only 1.5% of the cases. The main cause of death was cancer (59% of all deaths), with 42% due to CRC and 21% due to duodenal cancer. One patient died from thyroid cancer. The second and third most common causes of death were cardiovascular disease (13% of all deaths) and desmoid tumours (11% of all deaths), respectively.ConclusionExtending surveillance programmes to other cancers will not contribute significantly to the survival of patients with FAP.
Databáze: OpenAIRE