@article{362ce412c71f4c77b6cb39e97728b300,
title = "International consensus guidelines on surveillance for pancreatic cancer in chronic pancreatitis. Recommendations from the working group for the international consensus guidelines for chronic pancreatitis in collaboration with the International Association of Pancreatology, the American Pancreatic Association, the Japan Pancreas Society, and European Pancreatic Club",
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.",
keywords = "CT scan, EUS, Genetics, Markers, Risk factors, Surgery, Treatment",
author = "{for the Working Group for the International (IAP – APA – JPS – EPC) Consensus Guidelines for Chronic Pancreatitis} and William Greenhalf and Philippe L{\'e}vy and Thomas Gress and Vinciane Rebours and Brand, {Randall E.} and Steve Pandol and Suresh Chari and J{\o}rgensen, {Maiken Thyregod} and Julia Mayerle and Lerch, {Markus M.} and P{\'e}ter Hegyi and J{\"o}rg Kleeff and Castillo, {Carlos Fern{\'a}ndez del} and Shuiji Isaji and Tooru Shimosegawa and Andrea Sheel and Halloran, {Christopher M.} and Pramod Garg and Kyoichi Takaori and Besselink, {Marc G.} and Forsmark, {Chris E.} and Wilcox, {C. Mel} and Patrick Maisonneuve and Dhiraj Yadav and David Whitcomb and John Neoptolemos",
note = "Funding Information: The relative risk of pancreatic cancer in patients with cystic fibrosis and/or CFTR mutation carriers with/or without chronic pancreatitis is significantly increased but less so than that in patients with hereditary pancreatitis from PRSS1 mutations [ 26–30]. A study of 28,511 patients with cystic fibrosis from 1985 through 1992 in the United States and Canada identified 13 digestive tract cancers, during 164,764 person-years of follow-up, giving a ratio of observed to expected cancers of 6.5 (3.5–11.1) but there was only one case of pancreatic cancer [26]. As part of the same study the estimated numbers of patients with cystic fibrosis in 1992 as reported to the International Cystic Fibrosis Association, from 17 countries was approximately 24,500 with two cases of pancreatic cancer, giving an odds ratio of 31.5 with very wide confidence intervals (95% CI = 4.8–205) [26]. An analysis of nine patients with cystic fibrosis and pancreatic cancer identified from 1985 to 2006 reported from the USA, UK Germany, and Switzerland by Maisonneuve et al. was equivalent to an incidence of approximately 1 per 105 per year with a risk ratio of 5.3 (95% CI = 2.4–10.1) [28]. They concluded that although the estimated risk of pancreatic cancer in cystic fibrosis was “greater than in the general population, compared with other causes of mortality, the absolute risk of pancreatic cancer in patients with cystic fibrosis is negligible” [28]. A further study by Maisonneuve et al. followed 41,188 patients who received care at 250 cystic fibrosis care center programs in the USA from 1990 to 2009, with 344,114 patient-years of observation of non-transplanted patients and found only one pancreatic cancer, giving standardized incidence ratio of 0.8 (95% CI = 0.0–4.2) [53]. A recent meta-analysis six cohort studies with 99,925 patients with cystic fibrosis providing 544,695 person-years by Yamada et al., found a pooled standardized incidence ratio for pancreatic cancer of 6–18 (95% CI = 1.31–29.27) [54]. Maisonneuve et al. commented that “the low absolute number of patients with cancer, the lack of a well-established screening test, and the high burden of additional testing do not support the strong screening recommendations for pancreatobiliary cancers made by Yamada and colleagues” [55].JPN reports grants from NUCANA, Stiftung Deutsche Krebshilfe and Heidelberger Stiftung Chirurgie all outside of the submitted work; DCW is co-founder of Ariel Precision Medicine and may have equity. Publisher Copyright: {\textcopyright} 2020 IAP and EPC",
year = "2020",
month = jul,
doi = "10.1016/j.pan.2020.05.011",
language = "English (US)",
volume = "20",
pages = "910--918",
journal = "Pancreatology",
issn = "1424-3903",
publisher = "S. Karger AG",
number = "5",
}