{"title":"Characterization of Cholangiocarcinomas With Tubulocystic Morphology Associated With Biliary Adenofibroma or Biliary Adenofibroma-Like Lesions","authors":"Xiaoyan Liao , Diana Agostini-Vulaj , Rena X. Li , Xuchen Zhang","doi":"10.1016/j.modpat.2025.100815","DOIUrl":null,"url":null,"abstract":"<div><div>Hepatic biliary adenofibroma (BAF) is a benign neoplasm composed of tubuloglandular and microcystic structures within a fibrous stroma, resembling von Meyenburg complexes or ductal plate malformation (DPM). Intrahepatic cholangiocarcinoma (iCCA) with a DPM pattern (iCCA-DPM) is an established variant of iCCA, whereas adenofibroma-like tubulocystic carcinoma (AL-TCC) is a newly proposed iCCA variant associated with BAF-type lesions. We hypothesize that BAF, AL-TCC, and iCCA-DPM form a tumorigenic spectrum. Ten cases of surgically resected iCCAs with BAF (n = 1) or BAF-like (n = 9) lesions compatible with AL-TCC were analyzed and compared with 7 iCCA-DPM and 26 unspecified small duct iCCA (SD-iCCA). The AL-TCC cohort (6 women and 4 men) had a median age of 62 years. Tumors were often multifocal (70%), averaging 5.5 cm in size, with frequent lymphovascular invasion (40%), but no perineural invasion. Comparisons between AL-TCC and iCCA-DPM revealed no significant differences in age, sex, tumor size, focality, lymphovascular invasion, perineural invasion, or outcomes. When AL-TCC and iCCA-DPM were grouped together (n = 17) and compared with other unspecified SD-iCCA, the combined AL-TCC/iCCA-DPM cohort showed a stronger association with von Meyenburg complexes, biliary cysts, and/or bile duct adenomas (7/17 [41%] vs 0/26, <em>P</em> < .001), less perineural invasion (<em>P</em> = .027), more frequent ARID1A loss (11/17 [65%] vs 3/26 [12%], <em>P</em> < .001), and better patient outcomes (<em>P</em> = .036). Kaplan-Meier analysis revealed that ARID1A loss significantly improved patient survival (<em>P</em> = .046). In summary, AL-TCC with BAF or BAF-like lesions shares clinicopathologic and histogenetic characteristics with iCCA-DPM, suggesting that they are related and likely represent a continuum of tumorigenesis, distinct from other SD-iCCA.</div></div>","PeriodicalId":18706,"journal":{"name":"Modern Pathology","volume":"38 10","pages":"Article 100815"},"PeriodicalIF":5.5000,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Modern Pathology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0893395225001115","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"PATHOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Hepatic biliary adenofibroma (BAF) is a benign neoplasm composed of tubuloglandular and microcystic structures within a fibrous stroma, resembling von Meyenburg complexes or ductal plate malformation (DPM). Intrahepatic cholangiocarcinoma (iCCA) with a DPM pattern (iCCA-DPM) is an established variant of iCCA, whereas adenofibroma-like tubulocystic carcinoma (AL-TCC) is a newly proposed iCCA variant associated with BAF-type lesions. We hypothesize that BAF, AL-TCC, and iCCA-DPM form a tumorigenic spectrum. Ten cases of surgically resected iCCAs with BAF (n = 1) or BAF-like (n = 9) lesions compatible with AL-TCC were analyzed and compared with 7 iCCA-DPM and 26 unspecified small duct iCCA (SD-iCCA). The AL-TCC cohort (6 women and 4 men) had a median age of 62 years. Tumors were often multifocal (70%), averaging 5.5 cm in size, with frequent lymphovascular invasion (40%), but no perineural invasion. Comparisons between AL-TCC and iCCA-DPM revealed no significant differences in age, sex, tumor size, focality, lymphovascular invasion, perineural invasion, or outcomes. When AL-TCC and iCCA-DPM were grouped together (n = 17) and compared with other unspecified SD-iCCA, the combined AL-TCC/iCCA-DPM cohort showed a stronger association with von Meyenburg complexes, biliary cysts, and/or bile duct adenomas (7/17 [41%] vs 0/26, P < .001), less perineural invasion (P = .027), more frequent ARID1A loss (11/17 [65%] vs 3/26 [12%], P < .001), and better patient outcomes (P = .036). Kaplan-Meier analysis revealed that ARID1A loss significantly improved patient survival (P = .046). In summary, AL-TCC with BAF or BAF-like lesions shares clinicopathologic and histogenetic characteristics with iCCA-DPM, suggesting that they are related and likely represent a continuum of tumorigenesis, distinct from other SD-iCCA.
期刊介绍:
Modern Pathology, an international journal under the ownership of The United States & Canadian Academy of Pathology (USCAP), serves as an authoritative platform for publishing top-tier clinical and translational research studies in pathology.
Original manuscripts are the primary focus of Modern Pathology, complemented by impactful editorials, reviews, and practice guidelines covering all facets of precision diagnostics in human pathology. The journal's scope includes advancements in molecular diagnostics and genomic classifications of diseases, breakthroughs in immune-oncology, computational science, applied bioinformatics, and digital pathology.