Journal of hepato-biliary-pancreatic sciences · 2026
The Clinicopathological Characteristics and Prognosis of Cholangiolocarcinoma: A Multicenter Study With a Central Pathology Review.
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Ishii Takamichi, Makino Kenta, Hatano Etsuro, Komuta Mina, Akiba Jun, Sakamoto Yoshihiro, Ariizumi Shun-Ichi, Honda Goro, Hisaka Toru, Takeda Yutaka, Shimizu Akira, Yasuchika Kentaro, Mizuno Takashi, Asano Daisuke, Fujio Atsushi, Gotohda Naoto, Kitami Chie, Amaya Koji, Sadamori Hiroshi, Kawai Manabu, Nakakura Akiyoshi, Nakamura Masafumi, Endo Itaru
Аннотация
BACKGROUND: Cholangiolocarcinoma (CLC) is a rare liver cancer with ≥ 80% ductular configuration (DC). Given its rarity, its clinicopathological characteristics remain poorly understood, and its status as a distinct disease entity remains controversial. We conducted a nationwide multicenter study with centralized pathological review to investigate the features of CLC.
METHODS: We included patients with a postoperative diagnosis of CLC at 49 participating institutions (2005-2018). All cases were re-evaluated by centralized pathology according to the WHO classification.
RESULTS: Among 196 re-evaluated patients, 56 tumors were classified as CLC, 69 as intrahepatic cholangiocarcinoma (iCCA) with < 80% DC, 53 as small-duct type iCCA with DC, and 16 as mixed small- and large-duct type (SD/LD-iCCA with DC). CLC showed a low prevalence of viral hepatitis (23.2%), frequent arterial enhancement (83.9%), and characteristic apical EMA positivity. The median overall survival (OS) was 10.8 years, with 5- and 10-year OS rates of 85.0% and 73.7%, respectively, which were significantly better than those of SD/LD-iCCA with DC. Recurrence occurred in 38.2% of CLC cases with favorable outcomes following surgical resection.
CONCLUSIONS: CLC exhibits distinct pathological and prognostic features, including favorable long-term survival, supporting CLC as a distinct clinicopathological entity. Further molecular research is needed to refine the classification.
TRIAL REGISTRATION: This study was registered with the UMIN Clinical Trials Registry (UMIN000057919).
METHODS: We included patients with a postoperative diagnosis of CLC at 49 participating institutions (2005-2018). All cases were re-evaluated by centralized pathology according to the WHO classification.
RESULTS: Among 196 re-evaluated patients, 56 tumors were classified as CLC, 69 as intrahepatic cholangiocarcinoma (iCCA) with < 80% DC, 53 as small-duct type iCCA with DC, and 16 as mixed small- and large-duct type (SD/LD-iCCA with DC). CLC showed a low prevalence of viral hepatitis (23.2%), frequent arterial enhancement (83.9%), and characteristic apical EMA positivity. The median overall survival (OS) was 10.8 years, with 5- and 10-year OS rates of 85.0% and 73.7%, respectively, which were significantly better than those of SD/LD-iCCA with DC. Recurrence occurred in 38.2% of CLC cases with favorable outcomes following surgical resection.
CONCLUSIONS: CLC exhibits distinct pathological and prognostic features, including favorable long-term survival, supporting CLC as a distinct clinicopathological entity. Further molecular research is needed to refine the classification.
TRIAL REGISTRATION: This study was registered with the UMIN Clinical Trials Registry (UMIN000057919).