https://doi.org/10.1016/j.jcf.2026.08.012

The life expectancy of people with cystic fibrosis (PwCF) has improved dramatically, with a median predicted survival of 61 years (https://www.cff.org/medical-professionals/patient-registry). This progress is largely due to highly effective CFTR modulators such as elexacaftor, tezacaftor, ivacaftor (ETI, Trikafta) which correct the misfolding and gating defects caused by the common F508del mutation [1]. ETI therapy significantly improves pulmonary function and nutritional status in PwCF by enhancing CFTR processing and channel activity [1,2]. While these therapies have transformed CF care, their impact on long term complications-including malignancy risk-remain unclear.

PwCF face a disproportionately high risk of gastrointestinal cancers particularly pancreatic cancer, which occurs at younger ages and carries a poor prognosis [[3], [4], [5], [6]]. The underlying mechanisms are not fully understood but may involve chronic pancreatic inflammation....