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 |
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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 |
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