Risk of Gastrointestinal Cancers in Patients With Cystic Fibrosis

Key Points

  • Patients with cystic fibrosis were found to be at increased risk of GI cancers, with higher risk among those who had undergone lung transplantation.
  • Increased risks vs the general population were observed for small bowel, colon, biliary tract, and pancreatic cancers.

In a meta-analysis reported in The Lancet Oncology, Yamada et al found that patients with cystic fibrosis are at increased risk of gastrointestinal (GI) cancers vs the general population. Improved management of cystic fibrosis has led to increased life expectancy and increased incidence of malignancies among patients with cystic fibrosis.

Study Details

The study involved a systematic review of literature through August 2017, yielding 6 cohort studies including 99,925 patients (544,695 person-years) eligible for meta-analysis of GI cancer risk in patients with cystic fibrosis. The primary outcome measure was risk of GI and site-specific GI cancers in patients with cystic fibrosis compared with the general population.

Risk of GI Cancers

The overall risk of GI cancer was significantly higher in patients with cystic fibrosis vs the general population (pooled standardized incidence ratio [SIR] = 8.13, P < .0001). Patients who had undergone lung transplantation were at higher risk vs those who had not (pooled SIR = 21.13, P < .0001, vs pooled SIR = 4.18, P < .0001). Among site-specific cancers, patients were at increased risk for small bowel cancer (pooled SIR = 18.94, P < .0001), colon cancer (pooled SIR = 10.91, P < .0001), biliary tract cancer (pooled SIR = 17.87, P < .0001), and pancreatic cancer (pooled SIR = 6.18, P = .022).

The investigators concluded, “Our study suggests that patients with cystic fibrosis had a significantly increased risk of gastrointestinal cancer compared with the general population, including small bowel, colon, biliary tract, and pancreatic cancers. These findings highlight the need to develop individualised screening strategies for site-specific gastrointestinal cancers in patients with cystic fibrosis.”

The authors reported no funding for the study.

Atsushi Sakuraba, MD, Section of Gastroenterology, Hepatology and Nutrition, Department of Medicine, The University of Chicago, is the corresponding author for The Lancet Oncology article. 

The content in this post has not been reviewed by the American Society of Clinical Oncology, Inc. (ASCO®) and does not necessarily reflect the ideas and opinions of ASCO®.


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