scholarly journals Management of patients with increased risk for familial pancreatic cancer: updated recommendations from the International Cancer of the Pancreas Screening (CAPS) Consortium

Gut ◽  
2019 ◽  
Vol 69 (1) ◽  
pp. 7-17 ◽  
Author(s):  
Michael Goggins ◽  
Kasper Alexander Overbeek ◽  
Randall Brand ◽  
Sapna Syngal ◽  
Marco Del Chiaro ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
High Risk ◽  
Multidisciplinary Teams ◽  
Familial Pancreatic Cancer ◽  
High Grade ◽  
Degree Relative ◽  
Delphi Approach ◽  
Cancer Of The Pancreas ◽  
Increased Risk ◽  
Consensus Statements

Background and aimThe 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).MethodsA 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.ResultsConsensus 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.ConclusionsPancreatic 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.

Sa1350 – Updated International Cancer of the Pancreas Screening (CAPS) Consortium Guidelines on the Management of Patients with Increased Risk for Familial Pancreatic Cancer

2019 ◽  
Vol 156 (6) ◽  
pp. S-323
Author(s):  
Kasper A. Overbeek ◽  
Marcia I. Canto ◽  
Detlef K. Bartsch ◽  
Randall Brand ◽  
Alfredo Carrato ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Familial Pancreatic Cancer ◽  
Cancer Of The Pancreas ◽  
Increased Risk

Updated international Cancer of the Pancreas Screening (CAPS) consortium guidelines on the management of patients with increased risk for familial pancreatic cancer

Pancreatology ◽  
2019 ◽  
Vol 19 ◽  
pp. S56-S57
Author(s):  
Kasper Overbeek ◽  
Marcia Irene Canto ◽  
Detlef Bartsch ◽  
Randall Brand ◽  
Alfredo Carrato ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Familial Pancreatic Cancer ◽  
Cancer Of The Pancreas ◽  
Increased Risk

scholarly journals International Cancer of the Pancreas Screening (CAPS) Consortium summit on the management of patients with increased risk for familial pancreatic cancer

Gut ◽  
2012 ◽  
Vol 62 (3) ◽  
pp. 339-347 ◽  
Author(s):  
Marcia Irene Canto ◽  
Femme Harinck ◽  
Ralph H Hruban ◽  
George Johan Offerhaus ◽  
Jan-Werner Poley ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Familial Pancreatic Cancer ◽  
Cancer Of The Pancreas ◽  
Increased Risk

S2012 Prevalence of High Grade Neoplasia (HGN) in Familial Pancreatic Cancer (PC) Precursors:a Case-Control Study

2009 ◽  
Vol 136 (5) ◽  
pp. A-313
Author(s):  
Marcia I. Canto ◽  
Kieran Brune ◽  
Michael G. Goggins ◽  
Richard D. Schulick ◽  
Charles J. Yeo ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Case Control Study ◽  
Case Control ◽  
Familial Pancreatic Cancer ◽  
High Grade ◽  
Control Study

The Spanish familial pancreatic cancer registry panfam: Screening of high-risk individuals

Pancreatology ◽  
2015 ◽  
Vol 15 (3) ◽  
pp. S126
Author(s):  
Carmen Guillèn-Ponce ◽  
Julie Earl ◽  
Maria Teresa Salazar Lopez ◽  
Celia Calcedo ◽  
Reyes Ferreiro ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
High Risk ◽  
Cancer Registry ◽  
Familial Pancreatic Cancer

scholarly journals Genomic Features and Clinical Management of Patients with Hereditary Pancreatic Cancer Syndromes and Familial Pancreatic Cancer

2019 ◽  
Vol 20 (3) ◽  
pp. 561 ◽  
Author(s):  
Akihiro Ohmoto ◽  
Shinichi Yachida ◽  
Chigusa Morizane
Keyword(s):  
Pancreatic Cancer ◽  
Checkpoint Inhibitors ◽  
Susceptibility Gene ◽  
Treatment Strategies ◽  
Familial Pancreatic Cancer ◽  
Cancer Syndrome ◽  
Increased Risk ◽  
Clinical Benefits ◽  
Surveillance Programs ◽  
Cancer Syndromes

Pancreatic cancer (PC) is one of the most devastating malignancies; it has a 5-year survival rate of only 9%, and novel treatment strategies are urgently needed. While most PC cases occur sporadically, PC associated with hereditary syndromes or familial PC (FPC; defined as an individual having two or more first-degree relatives diagnosed with PC) accounts for about 10% of cases. Hereditary cancer syndromes associated with increased risk for PC include Peutz-Jeghers syndrome, hereditary pancreatitis, familial atypical multiple mole melanoma, familial adenomatous polyposis, Lynch syndrome and hereditary breast and ovarian cancer syndrome. Next-generation sequencing of FPC patients has uncovered new susceptibility genes such as PALB2 and ATM, which participate in homologous recombination repair, and further investigations are in progress. Previous studies have demonstrated that some sporadic cases that do not fulfil FPC criteria also harbor similar mutations, and so genomic testing based on family history might overlook some susceptibility gene carriers. There are no established screening procedures for high-risk unaffected cases, and it is not clear whether surveillance programs would have clinical benefits. In terms of treatment, poly (ADP-ribose) polymerase inhibitors for BRCA-mutated cases or immune checkpoint inhibitors for mismatch repair deficient cases are promising, and clinical trials of these agents are underway.

Cancer of the Pancreas: Clinical Outcome in 76 Cases

1986 ◽  
Vol 72 (6) ◽  
pp. 601-607 ◽  
Author(s):  
Vittorio Pugliese ◽  
Sebastiano Saccomanno ◽  
Luigina Bonelli ◽  
Daniela Barone ◽  
Massimo Conio ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
High Risk ◽  
Clinical Outcome ◽  
Clinical Presentation ◽  
High Sensitivity ◽  
Radical Resection ◽  
Final Diagnosis ◽  
Diagnostic Techniques ◽  
Tissue Diagnosis ◽  
Cancer Of The Pancreas

A final diagnosis of pancreatic cancer was established in 76 consecutive patients during 4 years. The clinical outcome was evaluated retrospectively, as well as clinical presentation and its impact on the rate of resectability. Even though the diagnostic techniques showed a high sensitivity, only 18.4% of patients had a radical resection performed. In 77.6% of the cases a tissue diagnosis had been obtained. However, in only 1/5 of them was the tissue proof obtained preoperatively. This review confirms that the survival of patients with pancreatic cancer is poor, with slight advantages in the few resectable cases. Therefore, an earlier diagnosis should be attempted in high-risk symptomatic patients, selected by means of nonaggressive tests and evaluated by means of more accurate diagnostic techniques, when suitable.

BRCA2 and predisposition to pancreatic and other cancers

2001 ◽  
Vol 3 (14) ◽  
pp. 1-10 ◽  
Author(s):  
Meghan A. Arnold ◽  
Michael Goggins
Keyword(s):  
Breast Cancer ◽  
Pancreatic Cancer ◽  
Early Detection ◽  
Pancreatic Adenocarcinoma ◽  
Germline Mutations ◽  
Familial Pancreatic Cancer ◽  
Pancreatic Carcinogenesis ◽  
Increased Risk ◽  
Late Event ◽  
Genetic Events

Pancreatic adenocarcinoma is a major cause of cancer deaths in the industrialised world. Recent work has focused on the genetics of pancreatic cancer with a goal of finding an early detection marker that might allow for greater rates of survival than are currently possible. The breast cancer 2 gene (BRCA2) is one of numerous genes implicated in familial pancreatic cancer. Carriers of germline mutations of the BRCA2 gene have an increased risk of several cancers, among them pancreatic adenocarcinoma. During pancreatic carcinogenesis, bi-allelic inactivation of BRCA2 occurs as a late event, suggesting that other genetic events must occur before neoplastic cells can tolerate loss of BRCA2.

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