Liao Xiaoyan, Zhang Xuchen
Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY, USA.
Department of Pathology, Yale University School of Medicine, New Haven, CT, USA.
J Clin Transl Pathol. 2024 Oct-Dec;4(4):163-167. doi: 10.14218/jctp.2024.00044. Epub 2024 Dec 24.
Hepatic biliary adenofibroma is an exceedingly rare biliary neoplasm that primarily affects adults. It typically presents as a solitary mass composed of low-grade microcystic and tubuloglandular bile duct structures, which are lined by low columnar to cuboidal non-mucin-producing biliary epithelium and supported by abundant fibrous stroma. Histologically, it resembles the Von Meyenburg complex but is much larger in size and often shows cytologic atypia. Although considered benign, emerging case studies and analyses suggest that biliary adenofibroma may serve as a precursor lesion to intrahepatic cholangiocarcinoma. However, its extreme rarity, coupled with an incompletely understood histogenesis, perpetuates diagnostic uncertainty and may lead to misclassification with other similar entities. This review consolidates the current understanding of the histopathological and molecular characteristics of biliary adenofibroma, highlights its differential diagnosis, explores its potential progression to cholangiocarcinoma, and discusses unresolved questions while proposing future research directions.
肝内胆管腺纤维瘤是一种极其罕见的胆管肿瘤,主要影响成年人。它通常表现为一个孤立的肿块,由低度微囊性和小管状腺性胆管结构组成,内衬低柱状至立方状不产生黏液的胆管上皮,并由丰富的纤维间质支持。组织学上,它类似于冯·迈恩伯格复合体,但体积大得多,且常显示细胞异型性。尽管被认为是良性的,但新出现的病例研究和分析表明,胆管腺纤维瘤可能是肝内胆管癌的前驱病变。然而,其极其罕见,加上组织发生学尚未完全了解,使得诊断存在不确定性,并可能导致与其他类似实体的误诊。本文综述巩固了目前对胆管腺纤维瘤组织病理学和分子特征的认识,强调了其鉴别诊断,探讨了其向胆管癌进展的可能性,讨论了未解决的问题,并提出了未来的研究方向。