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Zhang LM, Gu XW, Zhao HY, Gong X, Zhu YN. Thyroid-like intrahepatic cholangiocarcinoma, a rare subtype with distinct clinicopathological and molecular features: A case report. World J Gastrointest Oncol 2026; 18(12): 124848 [DOI: 10.4251/wjgo.124848]
Lian-Mei Zhang, Xiao-Wen Gu, Huan-Yu Zhao, Xue Gong, Ya-Ning Zhu, Department of Pathology, The Affiliated Huai’an No. 1 People’s Hospital of Nanjing Medical University, Huai’an 223300, Jiangsu Province, China
Author contributions: Zhang LM and Zhu YN designed the research; Zhang LM, Gu XW, Zhao HY, and Gong X performed the research; Zhang LM analyzed the data; Zhang LM wrote the paper; Zhu YN critically revised the manuscript and provided final approval. All authors have read and approved the final manuscript.
AI contribution statement: Portions of this manuscript were edited using AI tools solely for language refinement. The authors carefully reviewed and verified all AI-assisted outputs and take full responsibility for the scientific content of the manuscript.
Supported by Huaian Science and Technology Plan Project, No. HAB2024004 and Huai'an Natural Science Research Joint Program, No. HABL202241.
Informed consent statement: Informed written consent was obtained from the patient for publication of this case report and any accompanying images.
Conflict-of-interest statement: The authors have no conflicts of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Ya-Ning Zhu, Affiliate Associate Professor, Associate Chief Physician, Department of Pathology, The Affiliated Huai’an No. 1 People’s Hospital of Nanjing Medical University, No. 1 West Huanghe Road, Huaiyin District, Huai’an 223300, Jiangsu Province, China. 03nan.cy@163.com
Received: June 25, 2026 Revised: August 2, 2026 Accepted: October 8, 2026 Published online: December 15, 2026 Processing time: 137 Days and 22.7 Hours
Abstract
BACKGROUND
Thyroid-like intrahepatic cholangiocarcinoma (TLICC) is an extremely rare disease. Its histological features are similar to those of thyroid follicular tumors, which makes it prone to misdiagnosis. Previous studies have mainly focused on the clinical and pathological aspects, and the molecular characteristics remain unclear.
CASE SUMMARY
The literature review indicates that TLICC is more common in young women, has a stable biliary phenotype, and has a favorable postoperative prognosis. We report a case of a 37-year-old female with TLICC. Magnetic resonance imaging revealed a well-defined, lobulated mass approximately 7.2 centimeters in diameter in the right lobe of her liver. A pathological examination of the removed lesion revealed that it presented as multinodular, with a thin fibrous capsule. The tumor cells were arranged in a follicular, solid and island-like pattern, and the cell nuclei had a ground-glass appearance. Immunohistochemical analysis revealed that CK7, CK19, MOC31 and inhibin α were positive, while TTF-1, thyroid globulin, PAX8, HepPar1 and Glypican-3 were negative. The 120-gene panel identified potential pathogenic mutations in CHEK2, CDK12, and ERBB2, while no typical cholangiocarcinoma mutations were detected. The patient was followed up 12 months after the surgery and no disease progression was observed.
CONCLUSION
We report the first TLICC case with full molecular profiling. This tumor carries potential pathogenic genetic mutations in the CHEK2, CDK12 and ERBB2 genes, suggesting that TLICC may have unique genomic characteristics.
Core Tip: Thyroid-like intrahepatic cholangiocarcinoma is a rare variant that morphologically mimics thyroid follicular neoplasms. We report a case with comprehensive clinicopathological and molecular characterization. The tumor demonstrated positive staining for biliary markers and inhibin alpha, with negativity for thyroid markers. Next-generation sequencing revealed mutations in CHEK2, CDK12, and ERBB2, suggesting a distinct molecular profile from conventional cholangiocarcinoma. This case highlights the diagnostic challenges and the importance of integrating histology, immunohistochemistry, and molecular testing for accurate diagnosis.
