Intrahepatic cholangiocarcinoma (iCCA) represents a formidable malignancy originating from the epithelial lining and peribiliary glands of the intrahepatic bile ducts, ranging from the secondary branches to the minutest bile ductules. It is characterized by the expression of markers specific to cholangiocytes. This neoplasm constitutes 10-20% of all primary liver cancers6 and 20-30% of cholangiocarcinomas. In the context of China, a study revealed that among 26, 684 cases of surgically excised primary hepatic malignancies, hepatocellular carcinoma and iCCA comprised 90.2% and 8.2%, respectively. From 2006 to 2015, the age-standardized incidence rates of iCCA in China exhibited a modest increase from 2.0 to 2.2 per 100, 000, with a notable rise in the demographic aged over 65 years, escalating from 3.4 to 4.5 per 100, 000. iCCA is notorious for its aggressive nature, with a 5-year survival rate post-curative surgical resection languishing below 50%.
The clinicopathological distinctions between iCCA and extrahepatic cholangiocarcinoma are pronounced. Historically, the diagnosis of iCCA was hampered by a lack of refined diagnostic modalities, attributable to its heterogeneous histological presentations and ambiguous criteria for subtype classification. Nonetheless, the past decade has witnessed significant strides in the pathological understanding of iCCA, with several advancements incorporated into the fifth edition of the World Health Organization (WHO) Classification of Tumours of the Digestive System. Despite these advances, there remains a need for refinement in the histological classification criteria, differential diagnosis of histological variants, selection of immunohistochemical markers, and identification of molecular targets to enhance practical application. To date, no comprehensive guidelines delineating the pathological subtype diagnosis of iCCA have been established. Recognizing that a consensus on histological subtype diagnosis can streamline the pathological evaluation of iCCA, thereby furnishing a detailed pathological foundation for tailored clinical management, a panel of Chinese experts has collaboratively developed the present consensus on the pathological diagnosis of iCCA.
This expert consensus delineates nine recommendations aimed at standardizing and refining the diagnostic process for iCCA pathological subtypes, primarily anchored in the fifth edition of the WHO Classification of Tumours of the Digestive System.
The diagnosis of histological subtypes should be central to the routine pathological evaluation of iCCA. A critical aspect of this evaluation is distinguishing between large duct type and small duct type iCCAs. To aid in histological subtyping, it is advisable to optimize and utilize a combination of immunohistochemical marker panels. Additionally, the detection of molecular targets and immunotherapy biomarkers should be guided by the specific histological subtype of iCCA. Pathological reports should comprehensively detail the main pathobiological characteristics of iCCA. It is also recommended to avoid using the diagnostic term “intrahepatic cholangiocellular carcinoma” when referring to iCCA.
In the pathological reporting of iCCA, it is crucial to include features associated with the risks of postoperative recurrence and metastasis to inform clinical treatment strategies. These features should encompass the gross type, histological subtype, immunophenotype, differentiation grade, microvascular invasion, biological behavior, surgical margin status, pathological tumor–node–metastasis stage, background liver disease, and other pertinent information. To enhance the consistency and accuracy of iCCA pathological diagnoses, the adoption of a standardized template for pathological diagnostic reporting is strongly encouraged.
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https://www.xiahepublishing.com/2310-8819/JCTH-2023-00118
The study was recently published in the Journal of Clinical and Translational Hepatology.
The Journal of Clinical and Translational Hepatology (JCTH) is owned by the Second Affiliated Hospital of Chongqing Medical University and published by XIA & HE Publishing Inc. JCTH publishes high quality, peer reviewed studies in the translational and clinical human health sciences of liver diseases. JCTH has established high standards for publication of original research, which are characterized by a study’s novelty, quality, and ethical conduct in the scientific process as well as in the communication of the research findings. Each issue includes articles by leading authorities on topics in hepatology that are germane to the most current challenges in the field. Special features include reports on the latest advances in drug development and technology that are relevant to liver diseases. Regular features of JCTH also include editorials, correspondences and invited commentaries on rapidly progressing areas in hepatology. All articles published by JCTH, both solicited and unsolicited, must pass our rigorous peer review process.
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