A biannual pancreatic cancer surveillance program identifies a high prevalence of intraductal papillary mucinous neoplasms: accelerated malignant transformation in Peutz-Jeghers syndrome

Presenter: Yifan Wang MD | McGill University Background: Individuals with a family history of pancreatic adenocarcinoma (PC) or germline mutation in PC susceptibility gene are at higher risk developing PC. Surveillance high-risk individuals (HRIs) may allow earlier detection and its precursor lesions. Since advanced-stage PCs have been reported despite annual surveillance, shorter interval surveillance be justified. Methods: From 2013 to 2020, asymptomatic HRIs were enrolled program alternating between magnetic resonance imaging endoscopic ultrasound every 6 months. Results: Of 75 enrolled, 43 (57.3%) had (GS), 32 (42.7%) familial cancer (FPC) pedigree. Branch-duct intraductal papillary mucinous neoplasms (BD-IPMNs) identified 26 (34.7%) individuals, but only 2 developed progressive Despite biannual one patient Peutz-Jeghers syndrome (PJS) locally advanced arising from BD-IPMN. Whole genome sequencing this patient’s second PJS-associated the kindred revealed that both tumours driven by biallelic inactivation STK11 KRAS-independent manner. review 3,853 patients across two registries, we additional PJS Importantly, all 3 consistent malignant transformation an underlying BD-IPMN < The other lesion FPC underwent resection mixed-type IPMN which harboured polyclonal KRAS mutations. Furthermore, found colloid-type PCs, develop IPMNs, display COSMIC signature 17. Conclusion: Biannual identifies high rate BD-IPMNs HRIs, minority these progress Our findings suggest rapid transformation. In lower threshold for more effective than shortening intervals.