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Management of patients with increased risk for familial pancreatic cancer: updated recommendations from the International Cancer of the Pancreas Screening (CAPS) Consortium
  1. Michael Goggins1,
  2. Kasper Alexander Overbeek2,
  3. Randall Brand3,
  4. Sapna Syngal4,
  5. Marco Del Chiaro5,
  6. Detlef K Bartsch6,
  7. Claudio Bassi7,
  8. Alfredo Carrato8,
  9. James Farrell9,
  10. Elliot Fishman10,
  11. Paul Fockens11,
  12. Thomas M Gress12,
  13. Jeanin E van Hooft13,
  14. R H Hruban14,
  15. Fay Kastrinos15,16,
  16. Allison Klein17,
  17. Anne Marie Lennon18,
  18. Aimee Lucas19,
  19. Walter Park15,
  20. Anil Rustgi16,
  21. Diane Simeone20,
  22. Elena Stoffel21,
  23. Hans F A Vasen22,
  24. Djuna L Cahen2,
  25. Marcia Irene Canto18,
  26. Marco Bruno23
  27. International Cancer of the Pancreas Screening (CAPS) consortium
    1. 1Pathology, Medicine Oncology, Johns Hopkins University, Baltimore, Maryland, USA
    2. 2Gastroenterology and Hepatology, Erasmus Medical Center, Rotterdam, The Netherlands
    3. 3Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA
    4. 4GI Cancer Genetics and Prevention Program, Medical Oncology, Dana Farber Cancer Institute, Boston, Massachusetts, USA
    5. 5Department of Surgery, Division of Surgical Oncology, Denver, Colorado, USA
    6. 6Division of Visceral, Thoracic and Vascular Surgery, University of Marburg, Marburg, Germany
    7. 7Department of Surgey, University of Verona, Verona, Italy
    8. 8Medical Oncology, Hospital Ramón y Cajal, Madrid, Spain
    9. 9Medicine, Yale University School of Medicine, New Haven, Connecticut, USA
    10. 10The Russell H Morgan Department of Radiology and Radiological Science, Baltimore, Maryland, USA
    11. 11Department of Gastroenterology & Hepatology, Amsterdam Gastroenterology & Metabolism, Amsterdam, The Netherlands
    12. 12Gastroenterology, Endocrinology, Metabolism and Infectiology, University of Marburg, Marburg, Germany
    13. 13Gastroenterology and Hepatology, Amsterdam University Medical Centres, Amsterdam, The Netherlands
    14. 14Department of Pathology, Johns Hopkins University, Baltimore, Maryland, USA
    15. 15Division of Digestive and Liver Diseases, Columbia University Medical Center, New York City, New York, USA
    16. 16Division of Digestive and Liver Diseases, Columbia University, New York City, New York, USA
    17. 17Oncology, Johns Hopkins University, Baltimore, Maryland, USA
    18. 18Medicine, Johns Hopkins University, Baltimore, Maryland, USA
    19. 19Gastroenterology, Icahn School of Medicine at Mount Sinai, New York City, New York, USA
    20. 20New York University Medical Center, New York City, New York, USA
    21. 21University of Michigan, Ann Arbor, Michigan, USA
    22. 22Gastroenterology and Hepatology, Leiden University, Leiden, The Netherlands
    23. 23Gastoenterology and Hepatology, Erasmus University Rotterdam, Rotterdam, The Netherlands
    1. Correspondence to Dr Michael Goggins, Pathology, Medicine Oncology, Johns Hopkins University, Baltimore, Maryland, USA; mgoggins{at}jhmi.edu

    Abstract

    Background and aim The International Cancer of the Pancreas Screening Consortium met in 2018 to update its consensus recommendations for the management of individuals with increased risk of pancreatic cancer based on family history or germline mutation status (high-risk individuals).

    Methods A modified Delphi approach was employed to reach consensus among a multidisciplinary group of experts who voted on consensus statements. Consensus was considered reached if ≥75% agreed or disagreed.

    Results Consensus was reached on 55 statements. The main goals of surveillance (to identify high-grade dysplastic precursor lesions and T1N0M0 pancreatic cancer) remained unchanged. Experts agreed that for those with familial risk, surveillance should start no earlier than age 50 or 10 years earlier than the youngest relative with pancreatic cancer, but were split on whether to start at age 50 or 55. Germline ATM mutation carriers with one affected first-degree relative are now considered eligible for surveillance. Experts agreed that preferred surveillance tests are endoscopic ultrasound and MRI/magnetic retrograde cholangiopancreatography, but no consensus was reached on how to alternate these modalities. Annual surveillance is recommended in the absence of concerning lesions. Main areas of disagreement included if and how surveillance should be performed for hereditary pancreatitis, and the management of indeterminate lesions.

    Conclusions Pancreatic surveillance is recommended for selected high-risk individuals to detect early pancreatic cancer and its high-grade precursors, but should be performed in a research setting by multidisciplinary teams in centres with appropriate expertise. Until more evidence supporting these recommendations is available, the benefits, risks and costs of surveillance of pancreatic surveillance need additional evaluation.

    • early detection
    • familial pancreatic cancer
    • genetic predisposition
    • pancreatic ductal adenocarcinoma
    • surveillance

    https://doi.org/10.1136/gutjnl-2019-319352

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      Footnotes

      • Collaborators International Cancer of the Pancreas Screening (CAPS) consortium Paolo Giorgio Arcidiacono, Reiko Ashida, Margreet Ausems, Marc Besselink, Katharina Biermann, Bert Bonsing, Teri Brentnall, Amitabh Chak, Dayna Early, Carloz Fernandez-Del Castillo, Harold Frucht, Toru Furukawa, Steven Gallinger, Jennifer Geurts, Bas Groot Koerkamp, Pascal Hammel, Frederik Hes, Julio Iglesias-Garcia, Ihab Kamel, Masayuki Kitano, Günter Klöppel, Nanda Krak, Robert Kurtz, Richard Kwon, Jesse Lachter, Jeffrey Lee, Michael Levy, Giuseppe Malleo, Cheryl Meguid, Anirban Maitra, Daniel Margolis, Johan Offerhaus, Sara Olson, Salvatore Paiella, Walter Park, Gloria Petersen, Jan-Werner Poley, Francisco X Real, John Saltzman, Richard Schulick, Alina Stoita, Kyoichi Takaori, Masao Tanaka, Eric Tamm, Mark Topazian, Enrique Vazquez-Sequeiros, Frank Vleggaar, Wouter De Vos tot Nederveen Cappel, Charles Yeo, Martin Wasser, Anja Wagner, Michael Wallace, Christopher Wolfgang, Laura Wood.

      • Contributors The consensus meeting was initiated and organised by MG, MIC, MB, and DLC. The consensus study design was developed by KAO and revised and approved by MB, DLC, MG, and MIC. Relevant literature was collected and summarised by KAO. Presentations during the development workgroup meeting were given by MG, RB, MDC, EF, TMG, SS, MIC, and MB, and the discussions facilitated by JF and JEvH. All authors except KAO, and all previously mentioned study collaborators provided the data (votes) for this study. Data were collected and analysed by KAO. The results were critically reviewed by MB, DLC, MG, and MIC. The manuscript was drafted by MG, KAO, DLC, MIC, and MB. All authors approved the final manuscript.

      • Funding The consensus meeting was supported by NIH grant U01CA210170 and by a donation of “Kom in beweging tegen alvleesklierkanker”, “Living With Hope Foundation", and Hugh and Rachel Victor. MG is the Sol Goldman Professor of Pancreatic Cancer Research. AML is the Benjamin Baker Scholar.

      • Disclaimer JEvH received research funding from Abbott and Cook Medical; she is a consultant to Boston Scientific, Cook Medical, and Medtronics. DLC is a consultant to Tramedico. MB received research funding from Boston Scientific, Cook Medical, Pentax Medical, 3M; he is a consultant to Boston Scientific, Cook Medical, Pentax Medical, Mylan, MediRisk, and Medicom. PF is a consultant to Olympus, Cook Medical, Ethicon Endosurgery and received research funding from Boston Scientific. RB has received research funding from Immunovia and Freenome. MIC received research funding from Pentax C2 Cryoballoon and Endogastric Solutions. DS received research funding from Immunovia, Sanofi and Tempus; she is on the Scientific Advisory Board for Nybo Therapeutics, Interpace and Tyme.

      • Competing interests The authors disclose the following: JEvH received research funding from Abbott and Cook Medical; she is a consultant to Boston Scientific, Cook Medical, and Medtronics. DLC is a consultant to Tramedico. MB received research funding from Boston Scientific, Cook Medical, Pentax Medical, 3M; he is a consultant to Boston Scientific, Cook Medical, Pentax Medical, Mylan, MediRisk, and Medicom. PF is a consultant to Olympus, Cook Medical, Ethicon Endosurgery and received research funding from Boston Scientific. RB has received research funding from Immunovia and Freenome. MIC received research funding from Pentax C2 Cryoballoon and Endogastric Solutions. DS received research funding from Immunovia, Sanofi and Tempus; she is on the Scientific Advisory Board for Nybo Therapeutics, Interpace and Tyme.

      • Patient consent for publication Not required.

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

      • Data availability statement All data relevant to the study are included in the article.

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