探讨胆管癌相关多原发癌（CMPMs）患者临床病理特点、癌灶部位、肿瘤发生间隔时间及其与发病风险和预后的关系。

收集美国国立癌症研究所监测、流行病学和结果数据库（SEER）1973年至2014年间确诊为胆管癌的38 550例患者临床病理及随访资料。计算CMPMs标准化发病率（SIR）及其95%CI。采用SIR比较首发胆管癌后不同肿瘤的发病率，及其在不同年龄段及不同诊断时间间隔的发病率。比较CMPMs发病间隔时间对总体生存的影响。

单发胆管癌38 082例，CMPMs 468例，发病率1.2%。胆管癌后发生小肠、胰腺、肝及胆道肿瘤的风险较正常人群明显增加（SIR=5.97，1.58，2.28；95%CI：3.09～10.44，1.01～2.35，1.49～3.37；P<0.05）。首发年龄在30～49岁及50～59岁的患者发生其他肿瘤的风险明显高于正常人群（SIR=4.00，1.67；95%CI: 2.51～6.06，1.25～3.19；P<0.05）。胆管癌术后5～10年患者发生其他肿瘤的风险明显高于正常人群（SIR=1.30；95%CI：1.08～1.55；P<0.05）。胆管癌患者生存期随CMPMs发生的间隔时间增加而延长，尤其是胆管癌术后5年发生多原发癌总体生存状况具有明显优势。

CMPMs检出率低，易漏诊。对在30～59岁确诊，术后5年以上的胆管癌患者,加强对消化道肿瘤的随访可能有助于识别CMPMs。患者生存期随多原发癌发生的间隔时间增加而延长。

To investigate the clinicopathological characteristics, location of tumors, interval time of new-onset tumor, and their relationship with the onset risk and prognosis of patients with cholangiocarcinoma-related multiple primary malignancies (CMPMs).

Clinicopathological and follow-up data of 38 550 patients diagnosed with cholangiocarcinoma from the Surveillance, Epidemiology and End Results (SEER) database of National Cancer Institute between 1973 and 2014 were collected. The standardized incidence rate (SIR) of CMPMs and the 95%CI were calculated. The incidence of different tumors, different age and different diagnostic interval time after first-onset cholangiocarcinoma were compared with SIR. The impact of different interval time of CMPMs upon the overall survival was compared.

A total of 38 082 cases were diagnosed with single cholangiocarcinoma and 468 cases were diagnosed with CMPMs, with a incidence of 1.2%. The onset risk of small intestinal, pancreas, liver, and biliary tract tumors after cholangiocarcinoma was significantly higher than that in normal population (SIR=5.97, 1.58, 2.28; 95%CI: 3.09-10.44, 1.01-2.35, 1.49-3.37; P<0.05). Patients aged 30-49 and 50-59 years had a higher risk of developing other tumors, compared with the normal population (SIR=4.00, 1.67; 95%CI: 2.51-6.06, 1.25-3.19; P<0.05). At 5-10 years after surgery, the risk of other tumors in patients with cholangiocarcinoma was significantly higher than that of normal population (SIR=1.30; 95%CI: 1.08-1.55; P<0.05). The survival time of patients with cholangiocarcinoma was prolonged along with the increase of interval time of onsets of CMPMs. In particular, the overall survival of patients with CMPMs at postoperative 5 years was significantly better.

The diagnostic rate of CMPMs is low and the risk of missed diagnosis is high. For patients diagnosed with cholangiocarcinoma between the age of 30-59 years and have underwent surgery for over 5 years, enhance the follow-up of gastrointestinal tumors may contribute to identifying CMPMs. The survival time is prolonged along with the increase of interval time of new-onset CMPMs.