The Clinicopathological Characteristics and Prognosis of Cholangiolocarcinoma: A Multicenter Study With a Central Pathology Review

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).

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Journal
Journal of Hepato-Biliary-Pancreatic Sciences
Published
2026-08-14
DOI
https://doi.org/10.1002/jhbp.70170
Primary Topic
Cholangiocarcinoma and Gallbladder Cancer Studies
Type
article
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0.00

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article

The Clinicopathological Characteristics and Prognosis of Cholangiolocarcinoma: A Multicenter Study With a Central Pathology Review

Akira Shimizu, Takamichi Ishii, Yutaka Takeda, Jun Akiba et al.
Journal of Hepato-Biliary-Pancreatic Sciences
Cholangiocarcinoma and Gallbladder Cancer Studies
article

The Clinicopathological Characteristics and Prognosis of Cholangiolocarcinoma: A Multicenter Study With a Central Pathology Review

Akira Shimizu, Takamichi Ishii, Yutaka Takeda, Jun Akiba, Daisuke Asano, Chie Kitami, Yoshihiro Sakamoto, Mina Komuta, Naoto Gotohda, Koji Amaya, Etsuro Hatano, Kenta Makino, Kentaro Yasuchika, 五郎 本田, Atsushi Fujio, Akiyoshi Nakakura, Hiroshi Sadamori, Itaru Endo, Masafumi Nakamura, Manabu Kawai, Takashi Mizuno, Toru Hisaka, Shun‐Ichi Ariizumi
article en

Abstract

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).

Journal of Hepato-Biliary-Pancreatic Sciences
Kurume University (JP), Nagaoka University (JP), Kyushu University (JP), Shinshu University (JP), Wakayama Medical University (JP), Tohoku University (JP), Kyoto University (JP), Nagaoka Institute of Design (JP), Toyama Prefectural Central Hospital (JP), Kansai Rosai Hospital (JP), Fukuyama City Hospital (JP), Tokyo Women's Medical University (JP), Kyorin University Hospital (JP), National Cancer Center Hospital East (JP), Japanese Red Cross Society Wakayama Medical Center (JP), Institute of Science Tokyo (JP), Nagoya University (JP), International University of Health and Welfare (JP), Yokohama City University (JP)
Japan Society for the Promotion of Science
Good health and well-being
Openalex Percentile: Top 8%
Cholangiocarcinoma and Gallbladder Cancer Studies
DOIOpen Access PDFOpenAlex
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