Maisonneuve Patrick, FitzSimmons Stacey C, Neglia Joseph P, Campbell Preston W, Lowenfels Albert B
Division of Epidemiology and Biostatistics, European Institute of Oncology, Milan, Italy.
J Natl Cancer Inst. 2003 Mar 5;95(5):381-7. doi: 10.1093/jnci/95.5.381.
Cancer in patients with cystic fibrosis (CF), the most common genetic disorder in Caucasians, has been a rare event. However, more patients now reach adulthood, and more patients undergo organ transplantation-factors associated with an increased cancer risk. Our aim was to assess the risk of cancer in nontransplanted and transplanted CF patients.
We followed 28 858 patients whose data were reported to the Cystic Fibrosis Foundation patient registry from 1990 through 1999 and compared the number of cancers observed in transplanted and nontransplanted patients to the number expected from population-based cancer incidence data. All statistical tests were two-sided.
In 202 999 person-years of observation of nontransplanted CF patients, 75 cancers were observed, but 69.7 were expected (standardized incidence ratio [SIR] = 1.1, 95% confidence interval [CI] = 0.8 to 1.4). Twenty-three digestive tract tumors were observed, but 4.5 were expected (SIR = 5.1, 95% CI = 3.2 to 7.6). More cancers than expected were observed of the small bowel, colon, and biliary tract but not of the stomach or rectum. We found that the deficit of non-digestive tract tumors was not statistically significant (52 observed versus 65.2 expected; SIR = 0.80, 95% CI = 0.6 to 1.0; P =.055). In 2725 person-years of observation of 1063 transplanted patients, 13 cancers were observed, but 2.05 were expected (SIR = 6.3, 95% CI = 3.4 to 10.8), and more digestive tract tumors (four observed versus 0.19 expected; SIR = 21.2, 95% CI = 5.8 to 54.2) and lymphomas (seven observed versus 0.16 expected; SIR = 44.0, 95% CI = 17.7 to 90.7) were observed than expected.
We observed an increased risk of digestive tract cancers among adult CF patients, particularly of the small bowel, colon, and biliary tract. This increased risk appeared to be more pronounced in patients who had had an organ transplantation.
囊性纤维化（CF）是白种人中最常见的遗传性疾病，CF患者患癌症一直是罕见事件。然而，现在有更多患者活到成年，并且更多患者接受器官移植，这些因素都与癌症风险增加相关。我们的目的是评估未接受移植和接受移植的CF患者的癌症风险。
我们对1990年至1999年向囊性纤维化基金会患者登记处报告数据的28858名患者进行了随访，并将移植患者和未移植患者中观察到的癌症数量与基于人群的癌症发病率数据预期的数量进行比较。所有统计检验均为双侧检验。
在对未移植CF患者的202999人年观察中，观察到75例癌症，但预期为69.7例（标准化发病率[SIR]=1.1，95%置信区间[CI]=0.8至1.4）。观察到23例消化道肿瘤，但预期为4.5例（SIR=5.1，95%CI=3.2至7.6）。小肠、结肠和胆道观察到的癌症多于预期，但胃或直肠未观察到。我们发现非消化道肿瘤的不足无统计学意义（观察到52例，预期为65.2例；SIR=0.80，95%CI=0.6至1.0；P=0.055）。在对1063例移植患者的2725人年观察中，观察到13例癌症，但预期为2.05例（SIR=6.3，95%CI=3.4至10.8），并且观察到的消化道肿瘤（观察到4例，预期为0.19例；SIR=21.2，95%CI=5.8至54.2）和淋巴瘤（观察到7例，预期为0.16例；SIR=44.0，95%CI=17.7至90.7）多于预期。
我们观察到成年CF患者消化道癌症风险增加，尤其是小肠、结肠和胆道。这种增加的风险在接受器官移植的患者中似乎更为明显。
