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. 2016 May;21(5):594-9.
doi: 10.1634/theoncologist.2015-0446. Epub 2016 Mar 21.

Forty-Year Trends in Cholangiocarcinoma Incidence in the U.S.: Intrahepatic Disease on the Rise

Supriya K Saha  1 , Andrew X Zhu  1 , Charles S Fuchs  2 , Gabriel A Brooks  3
Affiliations

Forty-Year Trends in Cholangiocarcinoma Incidence in the U.S.: Intrahepatic Disease on the Rise

Supriya K Saha et al. Oncologist. 2016 May.

Abstract
in English, Chinese

Background: Challenges in the diagnosis and classification of cholangiocarcinoma have made it difficult to quantify the true incidence of this highly aggressive malignancy.

Methods: We analyzed the Surveillance, Epidemiology, and End Results data to assess long-term trends in the age-standardized incidence of intrahepatic and extrahepatic cholangiocarcinoma between 1973 and 2012, correcting for systematic coding errors. Because intrahepatic cholangiocarcinoma (ICC) may frequently be misdiagnosed as cancer of unknown primary (CUP), we also analyzed trends in the incidence of CUP.

Results: Between 1973 and 2012, the reported U.S. incidence of ICC increased from 0.44 to 1.18 cases per 100,000, representing an annual percentage change (APC) of 2.30%; this trend has accelerated during the past decade to an APC of 4.36%. The incidence of extrahepatic cholangiocarcinoma increased modestly from 0.95 to 1.02 per 100,000 during the 40-year period (APC, 0.14%). The incidence of CUP with histologic features potentially consistent with cholangiocarcinoma decreased by 51% between 1973 and 2012 (APC, -1.87%), whereas the incidence of CUP with squamous or nonepithelial histologic features increased modestly (APC, 0.42%).

Conclusion: The recognized incidence of ICC in the U.S. continues to rise, whereas the incidence of ECC is stable. The incidence of CUP has fallen dramatically during the same time period.

Implications for practice: Clinical distinctions between cholangiocarcinoma (particularly intrahepatic cholangiocarcinoma [ICC]) and cancer of unknown primary (CUP) can be challenging. Recent discoveries have identified recurrent and potentially targetable genomic abnormalities in ICC, highlighting the importance of improving diagnosis. This study demonstrates that the incidence of ICC is increasing in the U.S., whereas the incidence of extrahepatic cholangiocarcinoma is stable. Concomitantly, the incidence of CUP has declined dramatically, suggesting that improved distinction between ICC and CUP may be a major driver of the increasing recognized incidence of ICC. The increasing incidence of ICC warrants further study of prevention and treatment approaches.

摘要

背景. 在胆管癌诊断和分类中存在的挑战使得这种高度侵袭性恶性肿瘤的真实发病率难以量化。

方法. 我们对监测、流行病学及预后 (SEER) 项目中的数据进行了分析, 评估1973-2012年间肝内和肝外胆管癌年龄标化发病率的长期变化趋势 (根据系统编码误差校正) 。由于肝内胆管癌 (ICC) 常可能被误诊为原发部位不明的癌症 (CUP), 我们还对 CUP 发病率的变化趋势进行了分析。

结果. 1973-2012 年间, 美国 ICC 发病率从 0.44/10 万人增长到 1.18/10 万人, 相当于年变化率 (APC) 为 2.30%。这一趋势在过去十年间进一步加剧, APC 提高至 4.36%。肝外胆管癌在 40 年间从 0.95/10 万人略增加到 1.02/10 万人 (APC: 0.14%)。 1973-2012 年间, 组织学特征可能与胆管癌一致的 CUP 发病率下降了 51% (APC: -1.87%), 而鳞癌或非上皮性组织学特征的 CUP 的发病率则略有升高 (APC: 0.42%)。

结论. 在美国, 确诊的ICC发病率持续升高, 而ECC发病率保持稳定。而相同时期的CUP发病率则呈现大幅下降。The Oncologist 2016;21:594–599

对临床实践的提示: 对胆管癌[尤其是肝内胆管癌 (ICC) ]与原发部位不明的癌症 (CUP) 进行临床鉴别富有挑战性。 ICC 中近期识别出来的的复发和潜在靶位基因组异常强调了改进诊断的重要性。本研究证实美国的 ICC 发病率在持续升高, 而肝外胆管癌的发病率保持稳定。同一时期, CUP 的发病率则大幅下降, 提示 ICC 与 CUP 鉴别的进步可能是确诊的 ICC 发病率升高的主要驱动因素。 ICC 持续升高的发病率提示有必要针对其预防和治疗手段开展进一步的研究。

Keywords: Cancer of unknown primary; Epidemiology; Extrahepatic cholangiocarcinoma; Incidence; Intrahepatic cholangiocarcinoma; Trend.

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Conflict of interest statement

Disclosures of potential conflicts of interest may be found at the end of this article.

Figures

Figure 1.
Figure 1.
Age-adjusted incidence of intrahepatic cholangiocarcinoma and extrahepatic cholangiocarcinoma, 1973–2012. Abbreviations: ECC, extrahepatic cholangiocarcinoma; ICC, intrahepatic cholangiocarcinoma.
Figure 2.
Figure 2.
Age distribution of ICC and ECC. (A): Median age at diagnosis by era. (B): Percentage of patients diagnosed with ICC or ECC within the indicated age range. Abbreviations: ECC, extrahepatic cholangiocarcinoma; ICC, intrahepatic cholangiocarcinoma.
Figure 3.
Figure 3.
Stage distribution of ICC and ECC, 2004–2010. Data do not include cases with missing data for cancer stage. Abbreviations: ECC, extrahepatic cholangiocarcinoma; ICC, intrahepatic cholangiocarcinoma.
Figure 4.
Figure 4.
Relative incidence of ICC and ECC by gender, race, and ethnicity, 1993–2012. (A): Relative incidence (IRR), females relative to males. (B): Relative incidence, Hispanic relative to non-Hispanic ethnicity. (C): Relative incidence Asian/Pacific Islanders and blacks relative to whites. Abbreviations: ECC, extrahepatic cholangiocarcinoma; ICC, intrahepatic cholangiocarcinoma; IRR, incidence rate ratio; PI, Pacific Islander.
Figure 5.
Figure 5.
Age-adjusted incidence of CUP, stratified by histology codes potentially consistent versus not consistent with with cholangiocarcinoma, 1973–2012. Abbreviations: CC, cholangiocarcinoma; CUP, cancer of unknown primary.
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References

    1. Khan SA, Emadossadaty S, Ladep NG, et al. Rising trends in cholangiocarcinoma: is the ICD classification system misleading us? J Hepatol. 2012;56:848–854. - PubMed
    1. Khan SA, Thomas HC, Davidson BR, et al. Cholangiocarcinoma. Lancet. 2005;366:1303–1314. - PubMed
    1. Patel T. Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States. Hepatology. 2001;33:1353–1357. - PubMed
    1. Patel T. Worldwide trends in mortality from biliary tract malignancies. BMC Cancer. 2002;2:10. - PMC - PubMed
    1. Shaib YH, Davila JA, McGlynn K, et al. Rising incidence of intrahepatic cholangiocarcinoma in the United States: a true increase? J Hepatol. 2004;40:472–477. - PubMed

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