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Patients with cystic fibrosis have a significantly greater risk for developing cancers of the gastrointestinal tract compared with the general population, results of a systematic review and meta-analysis indicate.
Among persons with cystic fibrosis (CF) the standardized incidence ratio (SIR) for any gastrointestinal cancer compared with the general population was 8.13 (P less than .0001). Patients with CF were at significantly elevated risk for cancers of the small bowel, colon, biliary tract, and pancreas, reported Atsushi Sakuraba, MD, PhD, of the University of Chicago, and his colleagues.
“Additionally, our findings suggest that patients who had an organ transplant have a higher risk of developing gastrointestinal cancer than those who did not. Although further studies are needed to monitor gastrointestinal cancer incidence over time in patients with cystic fibrosis, the development of a screening strategy for gastrointestinal cancer in these patients is warranted,” they wrote in Lancet Oncology.
As patients with CF live longer because of improvements in therapies and management of comorbidities, their risks for cancer and other diseases will increase, the authors noted.
To get a more accurate estimate of the degree of risk than what smaller cohort studies could provide, the investigators conducted a systematic review and meta-analysis. They narrowed their search to six cohort studies including 99,925 patients with CF followed for a total of 5,444,695 person-years.
As noted, patients with CF overall had an eightfold higher risk for gastrointestinal cancers compared with the general population, and for the subgroup of patients who had undergone a lung transplant, the SIR was 21.13 compared with 4.18 for those with CF but no lung transplant (P less than .0001).
- 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.
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 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 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.
Patients with cystic fibrosis have a significantly greater risk for developing cancers of the gastrointestinal tract compared with the general population, results of a systematic review and meta-analysis indicate.
Among persons with cystic fibrosis (CF) the standardized incidence ratio (SIR) for any gastrointestinal cancer compared with the general population was 8.13 (P less than .0001). Patients with CF were at significantly elevated risk for cancers of the small bowel, colon, biliary tract, and pancreas, reported Atsushi Sakuraba, MD, PhD, of the University of Chicago, and his colleagues.
“Additionally, our findings suggest that patients who had an organ transplant have a higher risk of developing gastrointestinal cancer than those who did not. Although further studies are needed to monitor gastrointestinal cancer incidence over time in patients with cystic fibrosis, the development of a screening strategy for gastrointestinal cancer in these patients is warranted,” they wrote in Lancet Oncology.
As patients with CF live longer because of improvements in therapies and management of comorbidities, their risks for cancer and other diseases will increase, the authors noted.
To get a more accurate estimate of the degree of risk than what smaller cohort studies could provide, the investigators conducted a systematic review and meta-analysis. They narrowed their search to six cohort studies including 99,925 patients with CF followed for a total of 5,444,695 person-years.
As noted, patients with CF overall had an eightfold higher risk for gastrointestinal cancers compared with the general population, and for the subgroup of patients who had undergone a lung transplant, the SIR was 21.13 compared with 4.18 for those with CF but no lung transplant (P less than .0001).
- 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.
Patients with cystic fibrosis have a significantly greater risk for developing cancers of the gastrointestinal tract compared with the general population, results of a systematic review and meta-analysis indicate.
Among persons with cystic fibrosis (CF) the standardized incidence ratio (SIR) for any gastrointestinal cancer compared with the general population was 8.13 (P less than .0001). Patients with CF were at significantly elevated risk for cancers of the small bowel, colon, biliary tract, and pancreas, reported Atsushi Sakuraba, MD, PhD, of the University of Chicago, and his colleagues.
“Additionally, our findings suggest that patients who had an organ transplant have a higher risk of developing gastrointestinal cancer than those who did not. Although further studies are needed to monitor gastrointestinal cancer incidence over time in patients with cystic fibrosis, the development of a screening strategy for gastrointestinal cancer in these patients is warranted,” they wrote in Lancet Oncology.
As patients with CF live longer because of improvements in therapies and management of comorbidities, their risks for cancer and other diseases will increase, the authors noted.
To get a more accurate estimate of the degree of risk than what smaller cohort studies could provide, the investigators conducted a systematic review and meta-analysis. They narrowed their search to six cohort studies including 99,925 patients with CF followed for a total of 5,444,695 person-years.
As noted, patients with CF overall had an eightfold higher risk for gastrointestinal cancers compared with the general population, and for the subgroup of patients who had undergone a lung transplant, the SIR was 21.13 compared with 4.18 for those with CF but no lung transplant (P less than .0001).
- 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.
FROM THE LANCET ONCOLOGY
Key clinical point: Patients with cystic fibrosis, especially those who have received a lung transplant, are at increased risk for gastrointestinal tract cancers.
Major finding: The standard incidence ratio for GI cancers among patients with CF was 8.13 compared with the general population.
Study details: Systematic review and meta-analysis of published studies including 99,925 patients with CF followed for 5,444,695 person-years.
Disclosures: 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.