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Cancer genetics
Short Report
Extracolonic cancer risk in Dutch patients with APC (adenomatous polyposis coli)-associated polyposis
  1. Zeinab Ghorbanoghli1,2,
  2. Barbara AJ Bastiaansen3,
  3. Alexandra MJ Langers1,
  4. Fokko M Nagengast4,
  5. Jan-Werner Poley5,
  6. James CH Hardwick1,
  7. Jan J Koornstra6,
  8. Silvia Sanduleanu7,
  9. Wouter H de Vos tot Nederveen Cappel8,
  10. Ben JM Witteman9,
  11. H Morreau10,
  12. Evelien Dekker3,
  13. Hans FA Vasen1,2
  1. 1 Department of Gastroenterology & Hepatology, Leiden University Medical Center, Leiden, The Netherlands
  2. 2 Netherlands Foundation for the Detection of Hereditary Tumors, Leiden, The Netherlands
  3. 3 Department of Gastroenterology and Hepatology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands
  4. 4 Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands
  5. 5 Department of Gastroenterology and Hepatology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands
  6. 6 Department of Gastroenterology and Hepatology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands
  7. 7 Department of Internal Medicine, Division of Gastroenterology and Hepatology, Maastricht University Medical Center, Maastricht, The Netherlands
  8. 8 Department of Gastroenterology and Hepatology, Isala Clinics, Zwolle, The Netherlands
  9. 9 Department of Gastroenterology, Gelderse Vallei Hospital, Ede, The Netherlands
  10. 10 Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands
  1. Correspondence to Dr Hans FA Vasen, Department of Gastroenterology and Hepatology, Leiden University Medical Centre, Rijnsburgerweg 10, 2333 AA Leiden, The Netherlands; hfavasen{at}stoet.nl

Abstract

Background Screening 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.

Methods All 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.

Results A 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.

Conclusion Extending surveillance programmes to other cancers will not contribute significantly to the survival of patients with FAP.

  • Familial adenomatous polyposis
  • APC mutation
  • FAP
  • Extracolonic cancer

https://doi.org/10.1136/jmedgenet-2017-104545

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    Footnotes

    • Contributors Conception and design: ZG, HFAV.

      Provision of study materials or patients: BAJB, AMJL, FN, J-WP, JCHH, JJK, SS, WHVNC, BJMW, HM, ED.

      Collection and assembly of data: ZG, HFAV.

      Data analysis and interpretation: ZG, HFAV.

      Manuscript writing: All authors.

      Final approval of manuscript: All authors.

    • Funding Study supported by the Netherlands Foundation of Detection of Hereditary Tumours (NFDHT).

    • Competing interests None declared.

    • Patient consent Patients registered at the Netherlands Foundation for the Detection of Hereditary Tumors (StOET) sign a consent form approving the use of their medical data for research purposes upon registration.

    • Ethics approval Approved by boards of the Netherlands Foundation for the Detection of Hereditary Tumors (StOET) and the nationwide network and registry of histo- and cytopathology in the Netherlands (PALGA).

    • Provenance and peer review Not commissioned; externally peer reviewed.