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Patients with CF at increased risk for GI cancers

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CF: ‘A gastrointestinal cancer syndrome’

The emergence of gastrointestinal cancer as a clinical complication in adults with cystic fibrosis is a consequence of the substantial improvement in life expectancy. Novel CFTR modulator therapies, which correct the malfunctioning protein and increase its expression at the apical surface, might decrease the incidence of gastrointestinal cancers in patients with cystic fibrosis.

In conclusion, the meta-analysis by Yamada and colleagues shows that cystic fibrosis can be considered a gastrointestinal cancer syndrome, for which screening and surveillance protocols should be implemented. Oncologists and gastroenterologists managing patients with cystic fibrosis should consider the best methods for screening of the small bowel, biliary tract, pancreas, and colon to prevent gastrointestinal malignancies in these patients.

Mordechai Slae, MD, and Michael Wilschanski, MD, are with the Hadassah Medical Center in Jerusalem. These comments are condensed from their editorial in The Lancet Oncology.



  • The SIRs for site-specific gastrointestinal cancers were as follows:
  • Small bowel: SIR= 18.94 (P less than .0001).
  • Colon: SIR = 10.91 (P less than .0001).
  • Biliary tract: SIR = 17.87 (P less than .0001).
  • Pancreas: SIR = 6.18 (P = .022).

The investigators recommend screening for gastrointestinal cancers in patients with CF in general, and especially for patients who have undergone lung transplantation and are receiving immunosuppressive therapies.

Because of the elevated risk of pancreatic and biliary tract cancers, the authors propose a screening program including magnetic resonance cholangiopancreatography, endoscopic ultrasound, or abdominal ultrasound and measurement of cancer antigen 19-9.

The authors reported that the study had no funding source, and they declared no competing interests.

SOURCE: Yamada A et al. Lancet Oncol. 2018 Apr 26. doi: 10.1016/S1470-2045(18)30188-8.

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