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

Abstract

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.

Patient-reported burden of intensified surveillance and surgery in high-risk individuals under pancreatic cancer surveillance

“…After surgery, the pathological outcomes of the majority (80%) of individuals did not fulfill formal surveillance goals (seven resected with only low-grade dysplasia, one non-required resection for autoimmune pancreatitis). There is still no consensus amongst experts whether these cases should be considered a success of surveillance or as overtreatment [2]. Nevertheless, most patients deemed surgery to have been justified and all of them would likely or definitely choose to undergo surgery again.…”

“…Pancreatic cancer surveillance aims to detect pancreatic ductal adenocarcinoma (PDAC) or its precursors in a resectable stage. Because a non-invasive and accurate diagnostic test is lacking, and the incidence of PDAC in the general population is low [1], surveillance is only recommended for selected individuals with an increased lifetime risk [2]. For this group, magnetic resonance imaging (MRI) and/or endoscopic ultrasonography (EUS) at twelve-month intervals is advised [2].…”

“…Because a non-invasive and accurate diagnostic test is lacking, and the incidence of PDAC in the general population is low [1], surveillance is only recommended for selected individuals with an increased lifetime risk [2]. For this group, magnetic resonance imaging (MRI) and/or endoscopic ultrasonography (EUS) at twelve-month intervals is advised [2]. The goal is to detect stage one PDAC or, preferably, high-grade precursor lesions [2].…”

“…For this group, magnetic resonance imaging (MRI) and/or endoscopic ultrasonography (EUS) at twelve-month intervals is advised [2]. The goal is to detect stage one PDAC or, preferably, high-grade precursor lesions [2]. However, especially in small lesions, imaging techniques may not be able to distinguish malignancy and high-grade precursors from benign disease [3,4].…”

“…To prevent surgery for benign disease, individuals with lesions of unknown relevance are usually subjected to an intensified surveillance period, with shortened intervals of three or six months [2]. During this time, patients are burdened with the knowledge of having a lesion of unclear etiology, and with undergoing additional visits and investigations.…”