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World J Hepatol. Jul 27, 2023; 15(7): 897-903
Published online Jul 27, 2023. doi: 10.4254/wjh.v15.i7.897
Solid-Tubulocystic carcinoma: A new variant of intrahepatic cholangiocarcinoma
Iván A González, Wenyi Luo, Xuchen Zhang
Iván A González, Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, IN 46202, United States
Wenyi Luo, Xuchen Zhang, Department of Pathology, Yale University School of Medicine, New Haven, CT 06511, United States
Author contributions: González IA reviewed the literature and drafted the manuscript; Luo W provided some of the histological images and edited the manuscript; Zhang X provided overall intellectual input, reviewed the literature, and edited the final version of the manuscript; all authors approved the final version to be published.
Conflict-of-interest statement: The authors declare no conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Xuchen Zhang, MD, PhD, Department of Pathology, Yale University School of Medicine, 300 Cedar Street, New Haven, CT 06511, United States. xuchen.zhang@yale.edu
Received: March 17, 2023
Peer-review started: March 17, 2023
First decision: May 30, 2023
Revised: June 7, 2023
Accepted: June 13, 2023
Article in press: June 13, 2023
Published online: July 27, 2023
Processing time: 125 Days and 14.1 Hours
Core Tip

Core Tip: Solid-tubulocystic variant of intrahepatic cholangiocarcinoma (iCCA) is a recently recognized iCCA variant, which previously was termed as cholangioblastic iCCA. This new variant iCCA predominantly presents in young woman characterized by a heterogenous microscopic appearance with small to medium sized tumor cells with eosinophilic cytoplasm organized in solid, tubular, and cystic growth patterns. One of the defining features is the diffuse expression of inhibin. Recurrent NIPBL::NACC1 gene fusion has been identified in this iCCA variant. Compared to typical iCCAs, patients with this variant iCCA may have a better prognosis with 25% of the cases reported died of disease in 5 years.