Intraductal papillary mucinous neoplasms in high-risk individuals: incidence, growth rate, and malignancy risk

Kasper A Overbeek*, Brechtje D M Koopmann, the Dutch Familial Pancreatic Cancer Surveillance Study Group, Iris J M Levink, Matteo Tacelli, Nicole S Erler, Paolo Giorgio Arcidiacono, Margreet G E Ausems, Anja Wagner, Casper H van Eijck, Bas Groot Koerkamp, Olivier R Busch, Marc G Besselink, Manon van der Vlugt, Lydi M J W van Driel, Paul Fockens, Frank P Vleggaar, Jan-Werner Poley, Gabriele Capurso, Djuna L CahenMarco J Bruno

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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BACKGROUND AND AIMS: In high-risk individuals (HRIs), we aimed to assess the cumulative incidence of intraductal papillary mucinous neoplasms (IPMNs) and compare IPMN growth, neoplastic progression rate, and the value of growth as predictor for neoplastic progression to these in sporadic IPMNs.

METHODS: We performed annual surveillance of Dutch HRIs, involving carriers of germline pathogenic variants (PV) and PV-negative familial pancreatic cancer (FPC) kindreds. HRIs with IPMNs were compared to Italian individuals without familial risk under surveillance for sporadic IPMNs.

RESULTS: 457 HRIs were followed for 48 months (range 2-172); the estimated cumulative IPMN incidence was 46% (95%CI 28-64). In comparison to 442 controls, IPMNs in HRIs were more likely to grow ≥ 2.5 mm/year (31% versus 7%, P<0.001) and to develop worrisome features (32% versus 19%, P=0.010). PV carriers with IPMNs more often displayed neoplastic progression (n=3, 11%; versus n=6, 1%; P=0.011), while FPC kindreds did not (n=0, 0%; P=1.000). The malignancy risk in a PV carrier with an IPMN was 23% for growth rates ≥2.5 mm/year (n=13), 30% for ≥5 mm/year (n=10), and 60% for ≥10 mm/year (n=5).

CONCLUSIONS: The cumulative incidence of IPMNs in HRIs is higher than previously reported in the general population. Compared to sporadic IPMNs, they have an increased growth rate. PV carriers with IPMNs are suggested to be at a higher malignancy risk. Intensive follow-up should be considered for PV carriers with an IPMN growing ≥2.5 mm/year, and surgical resection for those growing ≥5 mm/year.

Original languageEnglish
Pages (from-to)62-71.e7
JournalClinical Gastroenterology and Hepatology
Issue number1
Early online date7 Apr 2023
Publication statusPublished - Jan 2024

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© 2024 AGA Institute


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