Tubulocystic Carcinoma of Bile Ducts: A Distinct Type of Cholangiocarcinoma Associated With Adenofibroma-type Lesions.

Autor:	Masetto F; Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona., Mafficini A; Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona.; ARC-Net Research Center, University and Hospital Trust of Verona., Saka B; Department of Pathology, Koç University Hospital and Koç University Research Center for Translational Medicine (KUTTAM), Istanbul, Turkey., Armutlu A; Department of Pathology, Koç University Hospital and Koç University Research Center for Translational Medicine (KUTTAM), Istanbul, Turkey., Chatterjee D; Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX., Jang KT; Department of Pathology and Translational Genomics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea., Zen Y; Institute of Liver Studies, King's College Hospital, London, UK., Navale P; Department of Pathology and Immunology, Washington University in St. Louis, St. Louis, MO., Fassan M; Department of Medicine (DIMED), Surgical Pathology Unit, University of Padua, and Veneto Institute of Oncology, IOV-IRCCS, Padua, Italy., Bacchi CE; Lab Bacchi, Botucatu, SP, Brazil., Mattiolo P; Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona., Simbolo M; Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona., Ruzzenente A; Department of Surgery, Dentistry, Gynecology and Pediatrics, Division of General and Hepatobiliary Surgery, University and Hospital Trust of Verona., Lawlor RT; ARC-Net Research Center, University and Hospital Trust of Verona.; Department of Engineering for Innovative Medicine (DIMI), University of Verona, Verona., Reid M; Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA., Basturk O; Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, NY., Adsay V; Department of Pathology, Koç University Hospital and Koç University Research Center for Translational Medicine (KUTTAM), Istanbul, Turkey., Scarpa A; Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona.; ARC-Net Research Center, University and Hospital Trust of Verona., Luchini C; Department of Diagnostics and Public Health, Section of Pathology, University and Hospital Trust of Verona.; ARC-Net Research Center, University and Hospital Trust of Verona.
Jazyk:	angličtina
Zdroj:	The American journal of surgical pathology [Am J Surg Pathol] 2024 Sep 01; Vol. 48 (9), pp. 1082-1092. Date of Electronic Publication: 2024 Jul 01.
DOI:	10.1097/PAS.0000000000002278
Abstrakt:	A type of cholangiocarcinoma (CCA) characterized by peculiar histologic patterns and underlying adenofibromatous lesions has been reported in the literature mostly as individual case reports. This study aims to further clarify the defining characteristics of this spectrum of lesions. Clinicopathologic analysis of 8 biliary tumors with tubulocystic architecture arising in the background of adenofibroma-type lesions was performed. Three of these were also investigated with next-generation sequencing with a 174 genes panel. The patients were 5 males and 3 females, with a mean age of 64.6. All tumors were intrahepatic except for one perihilar that protruded into soft tissues. The mean size was 4.4 cm. At histology, all cases showed a peculiar and cytologically bland tubulocystic pattern that closely resembled tubulocystic-type kidney cancers, including back-to-back microcystic units that formed relatively demarcated nodules, and occurring in the background of adenofibromatous lesions. One case showed perineural invasion by otherwise deceptively benign-appearing microcystic structures, one had areas transitioning to intraductal tubulopapillary neoplasm, and 3 cases harbored more conventional small-duct CCA foci. In those 3 cases, both the tubulocystic and conventional CCA components were investigated by next-generation sequencing separately, and they shared the molecular alterations, including recurrent mutations in chromatin remodeling genes, such as ARID1A , BAP1 , and PBRM1 , and the actionable FGFR2-MCU fusion gene. In the limited follow-up, all but one were alive and free of disease after surgical resection. In conclusion, we described a distinct entity of CCA with specific histo-molecular features, for which we propose the designation of tubulocystic carcinoma of bile ducts. Competing Interests: Conflicts of Interest and Source of Funding: A.S.: consulting and speaker bureau from: MSD, Incyte, Medica s.r.l., NTP. C.L.: consulting and speaker bureau from: MSD, Medica s.r.l., NTP. For the remaining authors, none were declared. (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)
Databáze:	MEDLINE
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