Article

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

Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.
Gut (Impact Factor: 13.32). 11/2012; 62(3). DOI: 10.1136/gutjnl-2012-303108
Source: PubMed

ABSTRACT BACKGROUND: Screening individuals at increased risk for pancreatic cancer (PC) detects early, potentially curable, pancreatic neoplasia. OBJECTIVE: To develop consortium statements on screening, surveillance and management of high-risk individuals with an inherited predisposition to PC. METHODS: A 49-expert multidisciplinary international consortium met to discuss pancreatic screening and vote on statements. Consensus was considered reached if ≥75% agreed or disagreed. RESULTS: There was excellent agreement that, to be successful, a screening programme should detect and treat T1N0M0 margin-negative PC and high-grade dysplastic precursor lesions (pancreatic intraepithelial neoplasia and intraductal papillary mucinous neoplasm). It was agreed that the following were candidates for screening: first-degree relatives (FDRs) of patients with PC from a familial PC kindred with at least two affected FDRs; patients with Peutz-Jeghers syndrome; and p16, BRCA2 and hereditary non-polyposis colorectal cancer (HNPCC) mutation carriers with ≥1 affected FDR. Consensus was not reached for the age to initiate screening or stop surveillance. It was agreed that initial screening should include endoscopic ultrasonography (EUS) and/or MRI/magnetic resonance cholangiopancreatography not CT or endoscopic retrograde cholangiopancreatography. There was no consensus on the need for EUS fine-needle aspiration to evaluate cysts. There was disagreement on optimal screening modalities and intervals for follow-up imaging. When surgery is recommended it should be performed at a high-volume centre. There was great disagreement as to which screening abnormalities were of sufficient concern to for surgery to be recommended. CONCLUSIONS: Screening is recommended for high-risk individuals, but more evidence is needed, particularly for how to manage patients with detected lesions. Screening and subsequent management should take place at high-volume centres with multidisciplinary teams, preferably within research protocols.

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    • "These high-risk candidates for screening include first degree relatives of patients with PDAC from familial kindred with at least two affected first-degree relatives , patients with PJS, and carriers of p16, BRCA2, or HNPCC mutations with at least one affected first-degree relative. The CAPS Consortium was not able to reach a consensus on the age to initiate screening or stop surveillance, as well as screening intervals, although agreement was made that initial screening should include EUS and/or MRI/MRCP, and not CT or ERCP (Canto et al., 2013). At this time, based on the current knowledge of pancreatic susceptibility genes, affected patients of FPC families should consider being tested for the most frequently inherited genetic defects identified in FPC, BRCA2, PALB2, and ATM germline mutations. "
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