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Original Investigation
March 15, 2017

Development and Validation of a Prognostic Score for Intrahepatic Cholangiocarcinoma

Author Affiliations
  • 1Department of Surgery, City of Hope National Medical Center, Duarte, California
JAMA Surg. Published online March 15, 2017. doi:10.1001/jamasurg.2017.0117
Key Points

Question  What are the prognostic determinants of overall survival among patients with intrahepatic cholangiocarcinoma undergoing liver resection?

Findings  In this evaluation of the California Cancer Registry and Office of Statewide Health, Planning, and Development database, records of 275 patients undergoing resection for intrahepatic cholangiocarcinoma were analyzed. A prognostic model based on the independent association of multifocality, extrahepatic extension, grade, nodal status, and age with overall survival was developed and validated.

Meaning  The prognostic score is an easy-to-use tool that allows accurate estimation of patient survival after hepatectomy for intrahepatic cholangiocarcinoma.


Importance  In patients with intrahepatic cholangiocarcinoma (ICC), the oncologic benefit of surgery and perioperative outcomes for large multifocal tumors or tumors with contiguous organ involvement remain to be defined.

Objectives  To develop and externally validate a simplified prognostic score for ICC and to determine perioperative outcomes for large multifocal ICCs or tumors with contiguous organ involvement.

Design, Setting, and Participants  This study of a contemporary cohort merged data from the California Cancer Registry (January 1, 2004, through December 31, 2011) and the Office of Statewide Health Planning and Development inpatient database. Clinicopathologic variables were compared between tumors that were intrahepatic, small (<7 cm), and solitary (ISS) and those that had extrahepatic extension and were large (≥7 cm) and multifocal (ELM). External validation of the prognostic model was performed using an independent data set from the National Cancer Institute’s Surveillance, Epidemiology, and End Results database from January 1, 2004, through December 31, 2013.

Main Outcomes and Measures  Patient overall survival after hepatectomy.

Results  A total of 275 patients (123 men [44.7%] and 152 women [55.3%]; median [interquartile range] age, 65 [55-72] years) met the inclusion criteria. No significant differences in overall complication rate (ISS, 48 [34.5%]; ELM, 37 [27.2%]; P = .19) and mortality rate (ISS, 10 [7.2%]; ELM, 6 [4.4%]; P = .32) were found. A multivariate Cox proportional hazards model demonstrated that multifocality, extrahepatic extension, grade, node positivity, and age greater than 60 years are independently associated with worse overall survival. These variables were used to develop the MEGNA prognostic score. The prognostic separation/discrimination index was improved with the MEGNA prognostic score (0.21; 95% CI, 0.11-0.33) compared with the staging systems of the American Joint Committee on Cancer sixth (0.17; 95% CI, 0.09-0.29) and seventh (0.18; 95% CI, 0.08-0.30) editions.

Conclusions and Relevance  The MEGNA prognostic score allows more accurate and superior estimation of patient survival after hepatectomy compared with current staging systems.