Characterization of Cholangiocarcinomas With Tubulocystic Morphology Associated With Biliary Adenofibroma or Biliary Adenofibroma-Like Lesions

IF 5.5 1区 医学 Q1 PATHOLOGY
Xiaoyan Liao , Diana Agostini-Vulaj , Rena X. Li , Xuchen Zhang
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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.
胆道腺纤维瘤或胆道腺纤维瘤样病变伴管状囊性胆管癌的特征分析。
肝胆道腺纤维瘤(BAF)是一种由纤维间质内的管状腺状和微囊状结构组成的良性肿瘤,类似于von Meyenburg复合物(VMC)或导管板畸形(DPM)。具有DPM模式的肝内胆管癌(iCCA) (iCCA-DPM)是iCCA的一种已知变体,而腺纤维瘤样管囊性癌(AL-TCC)是一种新提出的与baf型病变相关的iCCA变体。我们假设BAF、AL-TCC和iCCA-DPM形成了一个致瘤谱。我们分析了10例手术切除的与AL-TCC相容的BAF (n=1)或BAF样(n=9)病变的iCCA,并与7例iCCA- dpm和26例未明确的小管iCCA (SD-iCCA)进行了比较。AL-TCC队列(6名女性,4名男性)的中位年龄为62岁。肿瘤多为多灶性(70%),平均大小为5.5 cm,常伴有淋巴血管浸润(40%),但未见神经周围浸润。AL-TCC和iCCA-DPM的比较显示,在年龄、性别、肿瘤大小、病灶、淋巴血管侵犯、神经周围侵犯或预后方面没有显著差异。当AL-TCC和iCCA-DPM被分组时(n=17),与其他未指定的SD-iCCA相比,联合AL-TCC/iCCA-DPM队列显示与VMC、胆道囊肿和/或胆管腺瘤的相关性更强(7/17[41%]比0/26,P
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来源期刊
Modern Pathology
Modern Pathology 医学-病理学
CiteScore
14.30
自引率
2.70%
发文量
174
审稿时长
18 days
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.
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