William Greenhalf1, Philippe Lévy2, Thomas Gress3, Vinciane Rebours2, Randall E Brand4, Steve Pandol5, Suresh Chari6, Maiken Thyregod Jørgensen7, Julia Mayerle8, Markus M Lerch9, Péter Hegyi10, Jörg Kleeff11, Carlos Fernández-Del Castillo12, Shuiji Isaji13, Tooru Shimosegawa14, Andrea Sheel1, Christopher M Halloran1, Pramod Garg15, Kyoichi Takaori16, Marc G Besselink17, Chris E Forsmark18, C Mel Wilcox19, Patrick Maisonneuve20, Dhiraj Yadav21, David Whitcomb21, John Neoptolemos22. 1. Department of Molecular and Clinical Cancer Medicine, University of Liverpool, Liverpool, UK. 2. Service de Pancréatologie-Gastroentérologie, Pôle des Maladies de l'Appareil Digestif, DHU UNITY, Hôpital Beaujon, APHP, 92118 Clichy Cedex, and Université Paris 7, France. 3. Department of Gastroenterology, Endocrinology and Metabolism, University Hospital, Philipps-Universität Marburg, Marburg, Germany. 4. Division of Gastroenterology, Hepatology and Nutrition, Department of Internal Medicine, University of Pittsburgh Medical Center, 5200 Centre Avenue, Suite 409, Pittsburgh, PA, 15232, USA. 5. Cedars-Sinai Medical Center, Los Angeles, CA, United States Veterans Affairs Greater Los Angeles Healthcare System, Department of Medicine, University of California, Los Angeles, CA, USA. 6. University of Texas MD Anderson Cancer Center, Houston, TX, USA. 7. Syddansk University, Campusvej 55, 5230, Odense M, Denmark. 8. Department of Medicine II, University Hospital, LMU, Munich, Germany. 9. Department of Medicine A, University Medicine Greifswald, D-17475, Greifswald, Germany. 10. Institute for Translational Medicine &Department of Translational Medicine/1st Department of Medicine, Medical School, Pécs, H-7624, Hungary. 11. Department of Surgery, Martin-Luther University Halle-Wittenberg, Halle (Saale), Germany. 12. Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA. 13. Department of Surgery, Mie University Graduate School of Medicine, Japan. Electronic address: shujiisaji1@mac.com. 14. Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan. 15. Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India. 16. Department of Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan. 17. Department of Surgery, Amsterdam Gastroenterology Metabolism, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands. 18. Division of Gastroenterology, Hepatology, and Nutrition, University of Florida, Gainesville, FL, USA. 19. University of Alabama at Birmingham, USA. 20. Division of Epidemiology and Biostatistics, IEO European Institute of Oncology IRCCS, Milan, Italy. Electronic address: patrick.maisonneuve@ieo.it. 21. Department of Medicine University of Pittsburgh and UPMC, Pittsburgh, PA, USA. 22. Department of General Surgery, University of Heidelberg, Im Neuenheimer Feld 110, 69120, Heidelberg, Germany. Electronic address: john.neoptolemos@med.uni-heidelberg.de.
Abstract
BACKGROUND: Patients with chronic pancreatitis (CP) have an increased risk of pancreatic cancer. We present the international consensus guidelines for surveillance of pancreatic cancer in CP. METHODS: The international group evaluated 10 statements generated from evidence on 5 questions relating to pancreatic cancer in CP. The GRADE approach was used to evaluate the level of evidence available per statement. The working group voted on each statement for strength of agreement, using a nine-point Likert scale in order to calculate Cronbach's alpha reliability coefficient. RESULTS: In the following domains there was strong consensus: (1) the risk of pancreatic cancer in affected individuals with hereditary pancreatitis due to inherited PRSS1 mutations is high enough to justify surveillance; (2) the risk of pancreatic cancer in patients with CP associated with SPINK1 p. N34S is not high enough to justify surveillance; (3) surveillance should be undertaken in pancreatic specialist centers; (4) surveillance should only be introduced after the age of 40 years and stopped when the patient would no longer be suitable for surgical intervention. All patients with CP should be advised to lead a healthy lifestyle aimed at avoiding risk factors for progression of CP and pancreatic cancer. There was only moderate or weak agreement on the best methods of screening and surveillance in other types of environmental, familial and genetic forms of CP. CONCLUSIONS: Patients with inherited PRSS1 mutations should undergo surveillance for pancreatic cancer, but the best methods for cancer detection need further investigation.
BACKGROUND:Patients with chronic pancreatitis (CP) have an increased risk of pancreatic cancer. We present the international consensus guidelines for surveillance of pancreatic cancer in CP. METHODS: The international group evaluated 10 statements generated from evidence on 5 questions relating to pancreatic cancer in CP. The GRADE approach was used to evaluate the level of evidence available per statement. The working group voted on each statement for strength of agreement, using a nine-point Likert scale in order to calculate Cronbach's alpha reliability coefficient. RESULTS: In the following domains there was strong consensus: (1) the risk of pancreatic cancer in affected individuals with hereditary pancreatitis due to inherited PRSS1 mutations is high enough to justify surveillance; (2) the risk of pancreatic cancer in patients with CP associated with SPINK1 p. N34S is not high enough to justify surveillance; (3) surveillance should be undertaken in pancreatic specialist centers; (4) surveillance should only be introduced after the age of 40 years and stopped when the patient would no longer be suitable for surgical intervention. All patients with CP should be advised to lead a healthy lifestyle aimed at avoiding risk factors for progression of CP and pancreatic cancer. There was only moderate or weak agreement on the best methods of screening and surveillance in other types of environmental, familial and genetic forms of CP. CONCLUSIONS:Patients with inherited PRSS1 mutations should undergo surveillance for pancreatic cancer, but the best methods for cancer detection need further investigation.
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