• Stefan Buettner, Jeroen L A van Vugt, Faiz Gani, Bas Groot Koerkamp, Georgios Antonios Margonis, Cecilia G Ethun, George Poultsides, Thuy Tran, Kamran Idrees, Chelsea A Isom, Ryan C Fields, Bradley Krasnic... more k, Sharon M Weber, Ahmed Salem, Robert C G Martin, Charles Scoggins, Perry Shen, Harveshp D Mogal, Carl Schmidt, Eliza Beal, Ioannis Hatzaras, Rivfka Shenoy, Shishir K Maithel, Alfredo Guglielmi, Jan N M IJzermans, and Timothy M Pawlik. less
• Department of Surgery, Johns Hopkins Hospital, 600 N. Wolfe Street, Blalock 688, Baltimore, MD, 21287, USA.
• J. Gastrointest. Surg. 2016 Oct 1; 20 (10): 1716-24.

IntroductionAlthough widely used, the 7th edition American Joint Committee on Cancer (AJCC) staging system for perihilar cholangiocarcinoma (PHC) may be limited. Disease-specific nomograms have been proposed as a better means to predict long-term survival for individual patients. We sought to externally validate a recently proposed nomogram by Memorial Sloan Kettering Cancer Center (MSKCC) for PHC, as well as identify factors to improve the prediction of prognosis for patients with PHC.MethodsFour hundred seven patients who underwent surgery for PHC between 1988 and 2014 were identified using an international, multi-center database. Standard clinicopathologic and outcome data were collected. The predictive power of the AJCC staging system and nomogram were assessed.ResultsMedian survival was 24.4 months; 3- and 5-year survival was 37.2 and 20.8 %, respectively. The AJCC 7th edition staging system (C-index 0.570) and the recently proposed PHC nomogram (C-index 0.587) both performed poorly. A revised nomogram based on age, lymphovascular invasion, perineural invasion, and lymph node metastases performed better (C-index 0.682). The calibration plot of the revised PHC nomogram demonstrated good calibration.ConclusionThe 7th edition AJCC staging system and the MSKCC nomogram had a poor ability to predict long-term survival for individual patients with PHC. A revised nomogram provided more accurate prediction of survival, but will need to be externally validated.

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