Evaluating the Prognostic Impact of IDH Mutations in Intrahepatic Cholangiocarcinoma.

Publication Type Academic Article
Authors Harvey R, Gelfer R, Drill E, Balachandran V, D'Angelica M, Drebin J, Kingham T, Saadat L, Soares K, Wei A, Abou-Alfa G, Cercek A, Harding J, O'Reilly E, Doukas M, Homs M, Groot Koerkamp B, Jarnagin W
Journal JCO Precis Oncol
Volume 10
Issue 8
Pagination e2501261
Date Published 08/04/2026
ISSN 2473-4284
Keywords Isocitrate Dehydrogenase, Cholangiocarcinoma, Mutation, Bile Duct Neoplasms
Abstract PURPOSE: Isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) mutations are common in intrahepatic cholangiocarcinoma (ICC), but their prognostic value is unclear. Using a large data set, we assessed their impact in resected and nonresected ICC. METHODS: Adults from two medical centers (MSKCC and Erasmus) with ICC treated with curative-intent resection (resected) or managed nonoperatively (unresectable) who underwent next-generation sequencing were analyzed retrospectively. Kaplan-Meier and Cox regressions assessed the impact of IDH status on outcomes. RESULTS: Of the 795 patients analyzed, 25% had IDH1/2 mutations (IDHmut) and 43% underwent resection. Median overall survival (OS) of the cohort was 32 months in IDHmut and 28 months for IDHwt (P = .2). High-risk genetic alterations (TP53mut, KRASmut, and CDKN2Adel) were more frequent in IDH wild-type (IDHwt; odds ratio, 2.26; q < 0.001). OS was 19 months in patients with high-risk alterations versus 40 months in patients without (P < .001). In resected patients, recurrence-free survival (RFS) in IDHmut was 20 months versus 14 months for IDHwt (P = .018), and OS was 69 months versus 50 months, respectively (P = .2). However, after controlling for high-risk alterations, the potential benefit of IDHmut was no longer apparent (RFS: hazard ratio [HR], 0.78; P = .095; OS: HR, 0.88; P = .4). In unresectable IDHmut patients, progression-free survival was 9.4 months versus 9.1 months for IDHwt (P = .7), and OS was 22 months versus 18 months, respectively (P = .13). There remained no differences after controlling for high-risk alterations. IDH status was not a significant survival predictor in multivariable models. CONCLUSION: In this cohort of patients with ICC, IDHmut was not an independent predictor of survival, after controlling for high-risk alterations and clinical variables. IDH mutational status alone should, therefore, not be used to guide prognosis.
DOI 10.1200/PO-25-01261
PubMed ID 42550995
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