Journal of Liver Cancer

Review Articles

Combined hepatocellular-cholangiocarcinoma: a contemporary pathologic and molecular perspective: Taek Chung; J Liver Cancer. 2026;26(1):1-8. Published online March 9, 2026; DOI: https://doi.org/10.17998/jlc.2026.03.06

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Combined hepatocellular cholangiocarcinoma (cHCC-CCA) is a rare primary liver carcinoma characterized by the unequivocal coexistence of hepatocytic and cholangiocytic differentiation within a single tumor. Despite its low incidence, cHCC-CCA has received considerable attention because of its marked histologic heterogeneity, diagnostic challenges, and poorer clinical outcomes than conventional hepatocellular carcinoma. Historically, the biological nature of cHCC-CCA has been controversial, with competing hypotheses, including derivation from hepatic progenitor cells, collision of independent tumors, and transdifferentiation between hepatocytic and biliary lineages. Recent advances in genomic and transcriptomic profiling have substantially improved this understanding. Accumulating evidence indicates that most cHCC-CCAs arise from a common clonal origin and subsequently undergo divergent differentiation rather than representing true collision tumors. Transcriptomic analyses further demonstrate that cHCC-CCAs span a biological continuum between hepatocellular- and cholangiocytic-like states, with intermediate tumors characterized by lineage plasticity, activation of developmental pathways, and heterogeneous tumor microenvironments. This review provides a pathology- centered overview of cHCC-CCAs, summarizing the key histopathological features and the supportive role of immunohistochemistry, followed by an integrated discussion of recent genomic, transcriptomic, and immune profiling studies. Additionally, it highlights emerging applications of artificial intelligence and digital pathology, which may assist in biological stratification. Collectively, the current evidence supports viewing cHCC-CCA not as a single static entity, but as a spectrum of primary liver carcinomas unified by lineage plasticity, underscoring the importance of integrated pathological and multi-omics approaches for future classification and research.

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Curative Effect Evaluation of Targeted Therapy and Chemotherapy for Non-Resectable Combined Hepatocellular-Cholangiocarcinoma: A Systematic Review and Meta-Analysis
永豪林
Advances in Clinical Medicine.2026; 16(05): 920. CrossRef

Imaging differentiation of hepatocellular carcinoma, combined hepatocellular-cholangiocarcinoma, and intrahepatic cholangiocarcinoma: pitfalls and advances: Jaeseung Shin, Taek Chung, Sang Yun Ha, Hyungjin Rhee; J Liver Cancer. 2026;26(1):9-18. Published online March 5, 2026; DOI: https://doi.org/10.17998/jlc.2026.03.05

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Abstract PDF: Accurate non-invasive differentiation of primary liver cancers, such as hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (iCCA), and combined hepatocellular-cholangiocarcinoma (cHCC-CCA), is crucial for optimal management but challenging due to shared risk factors and overlapping imaging phenotypes. While the Liver Imaging Reporting and Data System category M effectively captures the classic targetoid appearance of large duct type iCCA, the small duct type frequently exhibits HCC-mimicking non-rim arterial phase hyperenhancement and non-peripheral washout, potentially compromising diagnostic specificity. Furthermore, cHCC-CCA presents a formidable diagnostic dilemma, existing on a continuous imaging spectrum that reflects its histologic dominance. This continuous imaging spectrum not only blurs radiologic distinctions but also complicates tissue sampling, limiting the diagnostic accuracy of core needle biopsies and highlighting the risk of misclassification. To enhance diagnostic clarity, this review highlights their key imaging hallmarks: while HCC typically shows non-rim arterial phase hyperenhancement (APHE) and non-peripheral washout, large duct iCCA displays a classic targetoid appearance with rim APHE and progressive central enhancement. Conversely, small duct iCCA often mimics HCC, and cHCC-CCA exhibits a variable spectrum depending on its predominant histologic component. Ultimately, overcoming these diagnostic pitfalls requires a rigorous, multidisciplinary approach that synthesizes imaging findings, serologic tumor markers, and clinical contexts.

The role of radiotherapy in the management of combined hepatocellular-cholangiocarcinoma: current evidence and future perspectives: Seo Hee Choi, Woong Sub Koom, Ik Jae Lee; J Liver Cancer. 2026;26(1):55-64. Published online March 5, 2026; DOI: https://doi.org/10.17998/jlc.2026.03.04

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Abstract PDF: Combined hepatocellular-cholangiocarcinoma (cHCC-CCA) is a rare and highly aggressive hybrid malignancy characterized by a poor prognosis and high recurrence rates due to its dual histological nature. In the absence of established standard-of-care protocols, clinical management strategies are frequently extrapolated from the guidelines for its components, hepatocellular carcinoma and intrahepatic cholangiocarcinoma (iCCA). This review evaluates the evolving role of radiotherapy (RT) as an integral part of the multidisciplinary care for cHCC-CCA. Adjuvant RT may be considered for patients exhibiting high-risk pathological features, such as positive or close resection margins, lymphovascular invasion, and perineural invasion. For unresectable disease unfeasible for surgery or transarterial therapies, definitive RT using intensified doses, analogous to iCCA protocols, is employed to improve local control. High-precision modalities, particularly particle therapies such as proton or carbon ion RT, are emphasized as preferred options for delivering ablative doses while minimizing toxicity and preserving functional liver reserve. Furthermore, preliminary clinical evidence suggests a potential synergy between RT and immune checkpoint inhibitors, with reported cases demonstrating complete responses or successful conversion to curative-intent resection. While current evidence remains limited to retrospective cohorts and case series, the strategic integration of precision RT offers a rational pathway for optimizing outcomes in cHCC-CCA, necessitating further prospective validation.

Contemporary overview of liver transplantation for intrahepatic cholangiocarcinoma and combined hepatocellular-cholangiocarcinoma: Hae Won Lee; J Liver Cancer. 2026;26(1):29-35. Published online March 5, 2026; DOI: https://doi.org/10.17998/jlc.2026.03.03

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Abstract PDF: Historically, intrahepatic cholangiocarcinoma (iCCA) and combined hepatocellular-cholangiocarcinoma (cHCC-CCA) were regarded as absolute contraindications for liver transplantation (LT) due to dismal outcomes characterized by high recurrence rates and poor long-term survival in early experiences. Consequently, these malignancies have been systematically excluded from standard transplant criteria for decades. However, the landscape of transplant oncology is undergoing a significant paradigm shift, driven by a deeper understanding of tumor biology and refined patient selection strategies. Recent multicenter retrospective studies have identified a distinct subgroup of patients-specifically those with “very early” iCCA in the setting of cirrhosis-who achieve excellent post-transplant outcomes comparable to those of hepatocellular carcinoma. This evidence has prompted major international societies to update their guidelines, cautiously opening the door for LT in this selected population. Conversely, cHCC-CCA remains a diagnostic and therapeutic challenge. This narrative review critically analyzes the pivotal data driving the current paradigm shift and synthesizes the latest clinical practice guidelines to provide a contemporary roadmap for the management of iCCA and cHCC-CCA in the transplant setting.

Systemic therapy for combined hepatocellular-cholangiocarcinoma: a comprehensive review of chemotherapy, immunotherapy, and targeted therapy: Jung Yong Hong, Dong Hyun Sinn, Sang Yun Ha; J Liver Cancer. 2026;26(1):36-44. Published online March 5, 2026; DOI: https://doi.org/10.17998/jlc.2026.03.02

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1 Citation

Abstract PDF

Combined hepatocellular-cholangiocarcinoma (cHCC-CCA) is a rare primary liver malignancy exhibiting both hepatocytic and cholangiocytic differentiation. Since the 2019 World Health Organization (WHO) reclassification, growing molecular and clinical evidence has reshaped our understanding of this entity. However, patients with cHCC-CCA have been systematically excluded from landmark clinical trials in both hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA), leaving clinicians without prospective evidence to guide treatment selection. This review comprehensively evaluates the current evidence on systemic therapy for advanced cHCC-CCA, encompassing cytotoxic chemotherapy, immune checkpoint inhibitors (ICIs), tyrosine kinase inhibitors, and molecularly targeted agents. Retrospective data indicate that gemcitabine plus platinum-based chemotherapy achieves the most consistent efficacy among conventional regimens, with median overall survival of 10-16 months. ICIs demonstrate objective response rates of 20-33% with durable responses in a subset of patients, supported by the finding that approximately 57% of cHCC-CCA tumors harbor an immune-high phenotype. Nearly 25% of tumors carry potentially actionable genomic alterations, including fibroblast growth factor receptor 2 (FGFR2) fusions, isocitrate dehydrogenase 1 (IDH1) mutations, and human epidermal growth factor receptor 2 (HER2) amplification. The molecular heterogeneity of cHCC-CCA, with tumors classifiable as HCC-like or CCA-like in approximately 75% of cases, provides a rational framework for personalized treatment selection. We propose an emerging molecular classification-based treatment algorithm and identify critical gaps requiring dedicated prospective investigation. For clinical settings where comprehensive genomic profiling is not feasible, we discuss a pragmatic surrogate-based approach using imaging characteristics and serum tumor markers to guide initial treatment selection. We also address post-progression treatment considerations, including phenotype-based regimen switching and the role of re-biopsy

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Curative Effect Evaluation of Targeted Therapy and Chemotherapy for Non-Resectable Combined Hepatocellular-Cholangiocarcinoma: A Systematic Review and Meta-Analysis
永豪林
Advances in Clinical Medicine.2026; 16(05): 920. CrossRef

Systemic treatment strategies for combined hepatocellular-cholangiocarcinoma: current evidence and future direction: Jeesun Yoon; J Liver Cancer. 2026;26(1):45-54. Published online February 26, 2026; DOI: https://doi.org/10.17998/jlc.2026.02.24

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Abstract PDF: Combined hepatocellular-cholangiocarcinoma (cHCC-CC) is a rare primary liver malignancy characterized by biphenotypic differentiation and marked biologic heterogeneity. Owing to its low incidence, diagnostic complexity, and lineage plasticity, standardized systemic treatment strategies remain undefined. Molecular and pathologic studies suggest a progenitor cell origin, with tumors exhibiting genomic, transcriptomic, and immunologic features overlapping with both hepatocellular carcinoma and intrahepatic cholangiocarcinoma. This heterogeneity contributes to variable therapeutic responsiveness and underscores the need for biologically informed treatment approaches. Current systemic treatment evidence is derived predominantly from retrospective analyses. Platinum-based cytotoxic chemotherapy has demonstrated modest but consistent clinical activity and remains the most commonly adopted palliative backbone. More recently, immunotherapy has shown encouraging anti-tumor activity, including combination strategies incorporating anti-angiogenic agents. Biomarker signals provide mechanistic rationale for immune-angiogenic therapeutic integration. Emerging platforms, including programmed cell death-1 (PD-1)/vascular endothelial growth factor (VEGF) bispecific antibodies, further expand the systemic treatment landscape. In parallel, multimodal strategies integrating locoregional interventions with systemic therapy are gaining traction, particularly for patients with liver-dominant disease. Despite these advances, prospective disease-specific trials remain lacking, and optimal therapeutic sequencing and patient selection strategies are yet to be defined. Future progress will depend on biomarker-driven trial design, incorporation of molecular lineage stratification, and rational combination approaches. A deeper understanding of the pathological and molecular architecture of cHCC-CC will be essential to establish optimized, disease-specific systemic treatment paradigms for this rare but clinically challenging malignancy.

Case Report

A rare case of IgG4-related sclerosing cholangitis followed by rapid subsequent diagnosis of cholangiocarcinoma: Gordon Hong, Amber Hussain, Eduardo Thadeu de Oliveira Correia, Akram Shalaby, Leonardo K. Bittencourt, Amit Mahipal, Lee M. Ocuin, Seth N. Sclair; J Liver Cancer. 2026;26(1):169-175. Published online December 17, 2025; DOI: https://doi.org/10.17998/jlc.2025.12.09

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Abstract PDF: Immunoglobulin G4 (IgG4)-related sclerosing cholangitis (IgG4-SC) is a rare condition with symptoms often mimicking malignancy, infection, or other autoimmune diseases. This case report describes the unique case of a 62-year-old male initially diagnosed with IgG4-SC, followed by subsequent diagnosis of cholangiocarcinoma. Biliary tract cancer in the setting of IgG4 related disease has been previously described; however, this patient course is novel as it encompasses the spectrum of challenges in IgG4-SC management, including diagnostic uncertainty, risk of infection with immunosuppressive agents, and development of malignancy diagnosed shortly following IgG4-SC diagnosis. We review the literature of management, outcomes, and malignancy risk and furthermore, highlight a promising recent therapy in treatment of IgG4 related disease, inebilizumab.

Review Article

New systemic treatment options for advanced cholangiocarcinoma: Valentina Zanuso, Giulia Tesini, Elena Valenzi, Lorenza Rimassa; J Liver Cancer. 2024;24(2):155-170. Published online August 8, 2024; DOI: https://doi.org/10.17998/jlc.2024.08.07

46,062 Views
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17 Citations

Abstract PDF

Cholangiocarcinoma (CCA) is a rare and aggressive cancer, mostly diagnosed at advanced or metastatic stage, at which point systemic treatment represents the only therapeutic option. Chemotherapy has been the backbone of advanced CCA treatment. More recently, immunotherapy has changed the therapeutic landscape, as immune checkpoint inhibitors have yielded the first improvement in survival and currently, the addition of either durvalumab or pembrolizumab to standard of care cisplatin plus gemcitabine represents the new first-line treatment option. However, the use of immunotherapy in subsequent lines has not demonstrated its efficacy and therefore, it is not approved, except for pembrolizumab in the selected microsatellite instability-high population. In addition, advances in comprehensive genomic profiling have led to the identification of targetable genetic alterations, such as isocitrate dehydrogenase 1 (IDH1), fibroblast growth factor receptor 2 (FGFR2), human epidermal growth factor receptor 2 (HER2), proto-oncogene B-Raf (BRAF), neurotrophic tropomyosin receptor kinase (NTRK), rearranged during transfection (RET), Kirsten rat sarcoma virus (KRAS), and mouse double minute 2 homolog (MDM2), thus favoring the development of a precision medicine approach in previously treated patients. Despite these advances, the use of molecularly driven agents is limited to a subgroup of patients. This review aims to provide an overview of the newly approved systemic therapies, the ongoing studies, and future research challenges in advanced CCA management.

Citations

Citations to this article as recorded by

Advancing systemic therapy for biliary tract cancer: current strategies and emerging paradigms
Khalil Choucair, Shadi Chamseddine, Asfar Azmi, Philip A. Philip
Frontiers in Oncology.2026;[Epub] CrossRef
Behavioral Medicine in Cholangiocarcinoma: Bridging the Gap Between Biology and Behavior
Leshui Wang, Yan Lu, Ye Qian, Xiao Wang, Yihuan Xu, Xinggang Guo, Wei Dong, Hui Liu, Susu Luo
Cancer Control.2026;[Epub] CrossRef
Genomic and transcriptomic signatures of sequential carcinogenesis from papillary neoplasm to biliary tract cancer
Taek Chung, Seungho Oh, Jeongsoo Won, Jiho Park, Jeong Eun Yoo, Ho Kyoung Hwang, Gi Hong Choi, Chang Moo Kang, Dai Hoon Han, Sangwoo Kim, Young Nyun Park
Journal of Hepatology.2025; 83(1): 119. CrossRef
A concise review of updated global guidelines for the management of hepatocellular carcinoma: 2017-2024
Hyunjae Shin, Su Jong Yu
Journal of Liver Cancer.2025; 25(1): 19. CrossRef
Modulating Wnt/β-catenin pathway activity to enhance chemosensitivity in cholangiocarcinoma
Kevin Delgado-Calvo, Luke Boulter, Oscar Briz, Aleksandra Rozyczko, Paula Olaizola, Jose J.G. Marin, Rocio I.R. Macias, Elisa Lozano
Biomedicine & Pharmacotherapy.2025; 188: 118225. CrossRef
Regorafenib plus modified gemcitabine-oxaliplatin in patients with advanced biliary tract cancer. The randomized phase Ib/II BREGO study
Jean-Frédéric Blanc, Mohamed Bouattour, Ludovic Gauthier, Emmanuel Deshayes, Sophie Guillemard, Yann Touchefeu, Fabienne Portales, Christophe Borg, Lobna Harguem, Rosine Guimbaud, Laurent Mineur, Marc Ychou, Thibault Mazard, Eric Assenat
The Oncologist.2025;[Epub] CrossRef
The new era of cholangiocarcinoma treatment: application of nano-based drug delivery systems
Paweena Dana, Prattana Tanyapanyachon, Saksorn Klibaim, Monthira Rattanatayarom, Walailuk Chonniyom, Nattika Saengkrit
Hepatoma Research.2025;[Epub] CrossRef
Diagnostic value of quantitative DWI and IVIM parameters in differentiating intrahepatic cholangiocarcinoma and hepatocellular carcinoma: a systematic review and meta-analysis
Saeed Mohammadzadeh, Alisa Mohebbi, Mehrad Zare, Faeze Salahshour, Afshin Mohammadi
Abdominal Radiology.2025; 51(3): 1244. CrossRef
Advanced cholangiocarcinoma with human epidermal growth factor receptor 2 (HER2) amplification treated with Trastuzumab deruxtecan (T-DXd): A case report
Xiaohui Bao, Zhi Chen, Jin Xiong, Zhenzhou Yang, Ni Zhang
Medicine.2025; 104(35): e44094. CrossRef
Validation of prognostic models for predicting postsurgical outcomes in intrahepatic cholangiocarcinoma patients using a multicenter cohort
Dong Hwan Kim, Sang Hyun Choi, Sehee Kim, Woohyung Lee, Hyung-Don Kim, Hyungjin Rhee, Eun-Suk Cho, Suk-Keu Yeom, Sumi Park, Seung Soo Lee, Mi-Suk Park
International Journal of Surgery.2025; 111(10): 7032. CrossRef
The Antibody–Drug Conjugate Sacituzumab Govitecan (IMMU-132) Represents a Potential Novel Therapeutic Strategy in Cholangiocarcinoma
Racha Hosni, Niklas Klümper, Christine Sanders, Sana Hosni, Vittorio Branchi, Alexander Semaan, Abdullah Alajati, Natalie Pelusi, Susanna S. Ng, Damian J. Ralser, Saif-Eldin Abedellatif, Hanno Matthaei, Jörg Kalff, Jasmitha Boovadira Poonacha, Veronika Lu
Molecular Cancer Therapeutics.2025; 24(11): 1775. CrossRef
Molecular Mechanisms and Therapeutic Perspectives of Gut Microbiota, Autophagy, and Apoptosis in Cholangiocarcinoma Pathophysiology
Viviana A. Ruiz-Pozo, Santiago Cadena-Ullauri, Patricia Guevara-Ramírez, Rafael Tamayo-Trujillo, Elius Paz-Cruz, Alejandro Cabrera-Andrade, Ana Karina Zambrano
International Journal of Molecular Sciences.2025; 26(20): 9949. CrossRef
Beyond futility: The history and potential of liver transplantation in cholangiocarcinoma
Lynn Affarah, Sreelakshmi Kotha, Philip Berry
World Journal of Transplantation.2025;[Epub] CrossRef
FGFR Aberrations in Solid Tumors: Mechanistic Insights and Clinical Translation of Targeted Therapies
Zijie He, Yizhen Chen, Genglin Li, Jintao Wang, Yuxin Wang, Pengjie Tu, Yangyun Huang, Lilan Zhao, Xiaojie Pan, Hengrui Liu, Wenshu Chen
Cancers.2025; 18(1): 89. CrossRef
Is 26S proteasome non-ATPase regulatory subunit 6 a potential molecular target for intrahepatic cholangiocarcinoma?
Yong-Zhi Zhuang, Li-Quan Tong, Xue-Ying Sun
World Journal of Hepatology.2024; 16(11): 1219. CrossRef
Imaging findings of intrahepatic cholangiocarcinoma for prognosis prediction and treatment decision-making: a narrative review
Jun Gu Kang, Taek Chung, Dong Kyu Kim, Hyungjin Rhee
The Ewha Medical Journal.2024;[Epub] CrossRef
Resectability and survival outcome in real world practice of 720 cholangiocarcinoma patients: intrahepatic, perihilar and distal cholangiocarcinoma.
Poowanai Sarkhampee, Weeris Ouransatien, Nithi Lertsawatvicha, Satsawat Chansitthichock, Paiwan Wattanarath
World Journal of Surgical Oncology.2024;[Epub] CrossRef

Case Report

Rare Clinical and Radiologic Case of Cholangiocarcinoma Mimicking Pyogenic Abscess, Hepatic Echinococcal Cysts, and Metastases: Si Hyeong Lee, Soo Hyung Ryu, Dong Hoon Lee, Won Eui Yoon, Tae Young Park, Hye Kyung Lee, Jeong Seop Moon; J Liver Cancer. 2020;20(2):173-176. Published online September 30, 2020; DOI: https://doi.org/10.17998/jlc.20.2.173

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2 Citations

Abstract PDF

Cholangiocarcinoma is a biliary carcinoma with a wide spectrum of imaging, histological, and clinical features. In immunocompromised patients, pyogenic abscesses are relatively common and an echinococcal hepatic cysts are very rare. The authors experienced a very rare case of cholangiocarcinoma showing multiple hypodense masses with wall enhancement mimicking pyogenic liver abscess, echinococcal hepatic cyst, and cystic metastases. An 83-year-old man, complaining of fatigue and poor oral intake, presented to our outpatient clinic. Abdominal computed tomography (CT) revealed multiple, variable-sized hypodense masses with peripheral rim enhancement throughout the liver. Dynamic liver magnetic resonance images also showed findings similar to those of a CT scan. We performed ultrasound-guided biopsy of the mass which revealed cholangiocarcinoma.

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Primary Cholangiocarcinoma of the Liver Presenting as a Complicated Hepatic Cyst: A Diagnostic Challenge
Chang Won Ha, Sang Deok Shin, Myung Ji Goh, Byeong Geun Song, Wonseok Kang, Dong Hyun Sinn, Geum-Youn Gwak, Yong-Han Paik, Moon Seok Choi, Joon Hyeok Lee
The Korean Journal of Gastroenterology.2025; 85(1): 83. CrossRef
Surgical strategy for incidental intrahepatic cholangiocarcinoma in terms of lymph node dissection
Sung Hyun Kim, Dai Hoon Han, Gi Hong Choi, Jin Sub Choi, Kyung Sik Kim
Journal of Gastrointestinal Surgery.2024; 28(6): 910. CrossRef

Original Article

Postoperative Chemoradiotherapy for R1 Resected Intrahepatic Cholangiocarcinoma: Kim, Kyung Su , Kim, Hwi Young , Kim, Kyubo , Yi, Nam Joon , Suh, Kyung Suk , Chie, Eui Kyu; J Liver Cancer. 2018;18(2):115-120. Published online September 30, 2018; DOI: https://doi.org/10.17998/jlc.18.2.115

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4 Citations

Abstract PDF

Background/Aims
To investigative the potential role of postoperative chemoradiotherapy (CCRT) after R1 resection of intrahepatic cholangiocarcinoma (IHCC).
Methods
Between January 2000 and December 2012, medical records of 18 patients who underwent curative surgery with R1 resection for IHCC were retrospectively reviewed.
Results
Median age was 68 years and 12 patients (66.7%) were male. Median tumor size was 5.0 cm (range, 2.2-11.0) and 12 patients (66.7%) had T3 or higher disease. Lymph nodes were involved in four patients (22.2%). Vascular invasion and perineural invasion were present in 10 (55.6%) and 12 patients (66.7%), respectively. Postoperative CCRT given with 5-fluorouracil or gemcitabine were delivered to 7 patients (38.9%). Median radiation dose was 50.4 Gy (range, 45-54). Univariate analysis showed that median loco-regional recurrence-free survival (LRRFS), progression-free survival (PFS) and overall survival (OS) were prolonged for patients treated with CCRT (median LRRFS; 5.6 months vs. not reached, P<0.001, median PFS; 5.6 vs. 8.3 months, P=0.047, median OS; 15.0 vs. 26.6 months, P=0.064).
Conclusions
Postoperative CCRT improved the loco-regional control and PFS in IHCC patients with R1 resection. Further study is warranted to validate the role of postoperative CCRT for these patients.

Citations

Citations to this article as recorded by

Prognostic Evaluation and Survival Prediction for Combined Hepatocellular-Cholangiocarcinoma Following Hepatectomy
Seok-Joo Chun, Yu Jung Jung, YoungRok Choi, Nam-Joon Yi, Kwang-Woong Lee, Kyung-Suk Suh, Kyoung Bun Lee, Hyun-Cheol Kang, Eui Kyu Chie, Kyung Su Kim
Cancer Research and Treatment.2025; 57(1): 229. CrossRef
Evolving role of radiation therapy in advanced/metastatic intrahepatic cholangiocarcinoma
Deepti Sharma, Babu Lal Meena
World Journal of Gastrointestinal Pharmacology and Therapeutics.2025;[Epub] CrossRef
Precision therapy for intrahepatic cholangiocarcinoma: A case report on adjuvant treatment in a recurrent patient after surgery and literature review
Bao Ying, Tao Tang, Li-Xing Zhang, Jian-Wei Xiong, Kai-Feng Zhao, Jia-Wei Li, Guo Wu
Oncology Letters.2023;[Epub] CrossRef
Stereotactic radiotherapy for intrahepatic cholangiocarcinoma
Aditya Borakati, Farid Froghi, Ricky H Bhogal, Vasileios K Mavroeidis
World Journal of Gastrointestinal Oncology.2022; 14(8): 1478. CrossRef

Case Reports

A Case of Combined Hepatocellular-cholangiocarcinoma Mimicking Pyogenic Liver Abscess: Seung Suk Baek, Eileen L. Yoon, Hyun-Jung Kim, Kyung Eun Bae, Kyeongmee Park, Won-choong Choi; J Liver Cancer. 2017;17(2):174-181. Published online September 30, 2017; DOI: https://doi.org/10.17998/jlc.17.2.174

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41 Downloads

Abstract PDF: Heterogeneous features of liver cancer can mimic liver abscess. Therefore it is essential to doublecheck tumor markers in the diagnosis of liver abscess. Herein, we report a case of combined hepatocellular-cholangiocarcinoma (cHC) occurred in an unrecognized chronic hepatitis B patient initially misdiagnosed as liver abscess. A 49-year old male initially presented with chill, right upper quadrant pain, and a liver mass. Mass showed peripheral enhancement in arterial phase of computed tomography, which was not typical for hepatocellular carcinoma (HCC). Strikingly elevated alpha-fetoprotein and fine needle aspirated pathology revealed HCC. Despite discordant image findings he was treated with transarterial chemoembolization. He was treated with sorafenib due to metastatic retrocaval lymphadenopathy afterwards. The mass presumed to be HCC progressed with sorafenib. It was surgically resected and he was finally confirmed as cHC. Discordant tumor markers with presumptive image findings should prompt the suspicion of rare type of primary liver cancer, the cHC.

A Case of Sarcomatoid Cholangiocarcinoma Which Developed at the Site Previously Treated by Transarterial Chemoembolization: Changkun Lee, Kyung Min Kang, Hae Lim Lee, Sung Won Lee, Heejeong Lee, Nam Ik Han; J Liver Cancer. 2017;17(1):100-104. Published online March 31, 2017; DOI: https://doi.org/10.17998/jlc.17.1.100

2,887 Views
15 Downloads

Abstract PDF: Intrahepatic sarcomatoid carcinoma is a rare tumor with poor prognosis due to its highly invasive and metastatic nature and difficulty for early detection. The most common form of intrahepatic sarcomatoid carcinoma is the sarcomatoid hepatocellular carcinoma, the development of which is usually associated with previous treatment for hepatocellular carcinoma. In contrast, sarcomatoid cholangiocarcinoma is extremely rare and results from spontaneous sarcomatoid transformation during the development of tumor. Here, we report a case of sarcomatoid cholangiocarcinoma, in a 58-year-old male, which developed at the site of previous treatment for hepatocellular carcinoma. A 9 × 7 cm sized tumor which had not been detected in the computed tomography exam 3 months before diagnosis was newly observed. The tumor rapidly progressed and the patient died only 31 days after the diagnosis.

Sarcomatoid Intrahepatic Cholangiocarcinoma: A Rare Case of Primary Liver Cancer: Doo Hyuck Lee, Kyu Hyung Han, Sun Young Ahn, Sang Sun Kim, Hyun Sung Shin, Ki Bae Bang, Jun Ho Choi, Suk Bae Kim, Won Ae Lee, il Han Song; J Liver Cancer. 2016;16(2):139-144. Published online September 30, 2016; DOI: https://doi.org/10.17998/jlc.16.2.139

3,051 Views
23 Downloads
3 Citations

Abstract PDF

Sarcomatoid carcinoma arising from intrahepatic cholangiocyte, an extremely rare primary liver cancer, has highly invasive and metastatic potential. The pathogenesis of this tumor is unclear, although histogenetic mechanisms, such as transdifferentiation/dedifferentiation (epithelialmesenchymal transition or metaplastic transformation), biphasic differentiation (combination and collision), and redifferentiation, might be suggested to explain the simultaneous coexistence of carcinoma and sarcoma components in the same tumor. Immunohistochemical staining might be necessary to differentiate whether sarcomatous component is originated from hepatocyte or cholangiocyte. We report a case of sarcomatoid intrahepatic cholangiocarcinoma in a 58 year-old man presenting as an incidentally detected liver mass on regular health examination, which was diagnosed by an application of immunohistochemical methods after surgical resection, with a review of the literature based on 9 cases reported in Korea.

Citations

Citations to this article as recorded by

Pathologic features and clinical treatment of sarcomatoid intrahepatic cholangiocarcinoma
Xiaoli Xie, Nannan Lai, Yuanyuan Yang, Jinwei Zhang, Jianmin Qin, Xia Sheng
Intractable & Rare Diseases Research.2023; 12(4): 267. CrossRef
Clinical diagnosis and treatment strategies for sarcomatoid intrahepatic cholangiocarcinoma
Xia Sheng, Jian-Min Qin
World Chinese Journal of Digestology.2022; 30(14): 614. CrossRef
Analysis of intrahepatic sarcomatoid cholangiocarcinoma: Experience from 11 cases within 17 years
Dong Kyun Kim, Bo Ra Kim, Jin Sook Jeong, Yang Hyun Baek
World Journal of Gastroenterology.2019; 25(5): 608. CrossRef

Review Article

Genomic Heterogeneity of Hepatocellular Carcinomas: Hyun Goo Woo; Journal of the Korean Liver Cancer Study Group. 2013;13(1):48-50. Published online February 28, 2013; DOI: https://doi.org/10.17998/jlc.13.1.48

1,722 Views
7 Downloads

Abstract PDF: Hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC) are major primary liver cancers in adults, comprising liver cancer spectrum. The existence of combined hepatocellular-cholangiocarcinoma (CHC), a histopathologic intermediate form between HCC and CC, suggests phenotypic overlap between these tumors. By applying an integrative oncogenomic approach, we identified a novel HCC subtype, i.e., cholangiocarcinoma-like HCC (CLHCC), which expressed cholangiocarcinoma-like traits (CC signature). In addition, we found that CLHCC coexpressed embryonic stem cell–like expression traits (ES signature) suggesting its derivation from bipotent hepatic progenitor cells. Further histopathological evaluation revealed a variant HCC with fibrous stromal component, i.e. scirrhous HCC, has CC-like genomic features, suggesting that the fibrous stromal component in HCC may contribute to the acquisition of CC-like gene expression trait in HCC. Our integrative analysis combining histopathological and genomic data would be a powerful approach to delineate the tumor heterogeneity.

Case Report

A One cm Sized Intrahepatic Cholangiocarcinoma in a Patient with Chronic Hepatiits B Misdiagnosed as Hepatocellular Carcinoma: Gene Hyun Bok, Soung Won Jeong, Jae Young Jang, Kwang Yeun Shim, Sae Hwan Lee, Sang Gyune Kim, Young Seok Kim, Hong Soo Kim, Boo Sung Kim, So Young Jin, Sung Sook Hong, Yong Jae Kim; Journal of the Korean Liver Cancer Study Group. 2012;12(1):23-27. Published online February 28, 2012

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2 Downloads

Abstract PDF: Morphologically, intrahepatic cholangiocarcinoma (ICC) presents as a parenchymal mass, and it is occasionally resectable and potentially curable. In some cases, differentiation from other hepatic neoplasms such as metastatic lesions and hepatocellular carcinoma (HCC) can be extremely difficult, both clinically and histologically, and definitive diagnosis often needs correlation with clinical and radiologic finding.Contrasted computed tomography (CT) is useful in the diagnosis of ICC and in determining the extent of tumor involvement. Although the majority of liver tumors can be diagnosed by modern imaging modalities such as contrast CT, some cases of ICC show tumor enhancement in the arterial phase the same as that in HCC, or a biliary dilatation without stenosis by intraductal tumor growth. Differences in these patterns of tumor enhancement and status of the bile ducts in ICC may also reflect differences in cellular characteristics, clinical features, and prognosis after treatment. We present a case of a small ICC showing similar clinical and radiologic characteristics to HCC.

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