Cancer risk in cystic fibrosis

A 20-year nationwide study from the United States

Patrick Maisonneuve, Bruce Marshall, Emily A. Knapp, Albert B. Lowenfels

Research output: Contribution to journalArticle

Abstract

BackgroundMany patients with cystic fibrosis (CF) now reach adulthood, at which time the risk of cancer is increased. The aim of this study was to determine cancer risks in nontransplanted and transplanted CF patients.MethodsFrom 1990 to 2009, we followed 41,188 patients who received care at one of the 250 CF care center programs in the United States and compared the observed number of cancers in nontransplanted and transplanted patients with that expected in the general US population.ResultsIn 344,114 patient-years of observation of nontransplanted patients, the overall cancer risk was similar to the background risk (standardized incidence ratio [SIR] = 1.1, 95% confidence interval [CI] = 1.0 to 1.3). However, we observed an elevated risk of digestive tract cancer (SIR = 3.5, 95% CI = 2.6 to 4.7) involving the esophago-gastric junction, biliary tract, small bowel, and colon. There was also an increased risk of testicular cancer (SIR = 1.7, 95% CI = 1.02 to 2.7) and lymphoid leukemia (SIR = 2.0, 95% CI = 1.2 to 3.1) and a decreased risk of malignant melanoma (SIR = 0.4, 95% CI = 0.2 to 0.9). In 8235 patient-years of observation of transplanted patients, 26 tumors were observed compared with 9.6 expected (SIR = 2.7, 95% CI = 1.8 to 3.9). The increased risk was particularly high for digestive tract cancers (SIR = 17.3, 95% CI = 10.7 to 26.5), with most cases arising in the bowel.ConclusionsThe overall burden of cancer in CF patients remains low; however they have an increased risk of digestive tract cancer, particularly following transplantation. They also have increased risk of lymphoid leukemia and testicular cancer, and decreased risk of melanoma.

Original languageEnglish (US)
Pages (from-to)122-129
Number of pages8
JournalJournal of the National Cancer Institute
Volume105
Issue number2
DOIs
StatePublished - Jan 16 2013
Externally publishedYes

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Cystic Fibrosis
Confidence Intervals
Neoplasms
Incidence
Lymphoid Leukemia
Gastrointestinal Tract
Testicular Neoplasms
Melanoma
Observation
Biliary Tract
Stomach
Colon
Transplantation

ASJC Scopus subject areas

  • Medicine(all)
  • Oncology
  • Cancer Research

Cite this

Cancer risk in cystic fibrosis : A 20-year nationwide study from the United States. / Maisonneuve, Patrick; Marshall, Bruce; Knapp, Emily A.; Lowenfels, Albert B.

In: Journal of the National Cancer Institute, Vol. 105, No. 2, 16.01.2013, p. 122-129.

Research output: Contribution to journalArticle

Maisonneuve, Patrick ; Marshall, Bruce ; Knapp, Emily A. ; Lowenfels, Albert B. / Cancer risk in cystic fibrosis : A 20-year nationwide study from the United States. In: Journal of the National Cancer Institute. 2013 ; Vol. 105, No. 2. pp. 122-129.
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AB - BackgroundMany patients with cystic fibrosis (CF) now reach adulthood, at which time the risk of cancer is increased. The aim of this study was to determine cancer risks in nontransplanted and transplanted CF patients.MethodsFrom 1990 to 2009, we followed 41,188 patients who received care at one of the 250 CF care center programs in the United States and compared the observed number of cancers in nontransplanted and transplanted patients with that expected in the general US population.ResultsIn 344,114 patient-years of observation of nontransplanted patients, the overall cancer risk was similar to the background risk (standardized incidence ratio [SIR] = 1.1, 95% confidence interval [CI] = 1.0 to 1.3). However, we observed an elevated risk of digestive tract cancer (SIR = 3.5, 95% CI = 2.6 to 4.7) involving the esophago-gastric junction, biliary tract, small bowel, and colon. There was also an increased risk of testicular cancer (SIR = 1.7, 95% CI = 1.02 to 2.7) and lymphoid leukemia (SIR = 2.0, 95% CI = 1.2 to 3.1) and a decreased risk of malignant melanoma (SIR = 0.4, 95% CI = 0.2 to 0.9). In 8235 patient-years of observation of transplanted patients, 26 tumors were observed compared with 9.6 expected (SIR = 2.7, 95% CI = 1.8 to 3.9). The increased risk was particularly high for digestive tract cancers (SIR = 17.3, 95% CI = 10.7 to 26.5), with most cases arising in the bowel.ConclusionsThe overall burden of cancer in CF patients remains low; however they have an increased risk of digestive tract cancer, particularly following transplantation. They also have increased risk of lymphoid leukemia and testicular cancer, and decreased risk of melanoma.

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