Cancer risk in non-transplanted and transplanted cystic fibrosis patients

P Maisonneuve Ing a, SC FitzSimmons PhD b, JP Neglia MD c, P Campbell PhD d, AB Lowenfels MD e

a Division of Epidemiology and Biostatistics, European Institute of Oncology, Milan, Italy b FitzSimmons and Associates, Epidemiology and Clinical Research, Bethesda, MD c Departments of Pediatrics and Epidemiology, University of Minnesota Schools of Medicine and Public Health, Minneapolis, MN d Cystic Fibrosis Foundation, Bethesda, MD e Department of Community and Preventive Medicine, New York Medical College, Valhalla, NY

AIM A previous report identified excess digestive cancers in patients with cystic fibrosis (CF), the commonest genetic disorder of whites. One third of CF patients now reach adulthood and transplantation had become a more common treatment modality of these patients. Our aim was to confirm and detail cancer risk in non-transplanted and in transplanted CF patients. METHODS We used data from 28,842 CF patients registered at the Cystic Fibrosis Foundation from 1990 through 1999 and compared the number of cancer observed from diagnosis of CF until transplantation and after transplantation to the respective expected numbers calculated from population-based cancer incidence data. RESULTS In non-transplanted CF patients, 75 cancers were observed and 69.7 expected during 202,999 person-years of observation (SIR=1.1; 0.8-1.4). We found an excess of digestive cancers (obs=24, exp=4.5, SIR=5.3 (3.4-7.9)), restricted to cancer of the small bowel, colon, biliary tract, liver, pancreas and esophagus, but not stomach or rectum. No excess of cancer was found at other sites, while a non-significant deficit of respiratory cancer reflect smoking abstinence in CF patients. In total 1,063 CF patients had been transplanted and 43 cancers developed in these patients after transplantation. Again, we observed an excess of digestive tumors (obs=4, exp=0.19) and of post transplant lymphoproliferative diseases (obs=38, exp=0.16). CONCLUSIONS Although the overall cancer risk in CF patients is similar to that of the general population, there is an increased risk of particular digestive tract cancers, enhanced after transplantation. Persistent or unexplained gastrointestinal symptoms in CF patients should be carefully investigated.

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Paper presented at the International Symposium on Predictive Oncology and Intervention Strategies; Paris, France; February 9 - 12, 2002; in the section on Risk Assessment, Part 2.