Liao Xiaoyan, Agostini-Vulaj Diana, Li Rena X, Zhang Xuchen
Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, New York.
Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, New York.
Mod Pathol. 2025 Jun 13;38(10):100815. doi: 10.1016/j.modpat.2025.100815.
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.
肝内胆管腺纤维瘤（BAF）是一种良性肿瘤，由纤维性间质内的小管状腺泡和微囊结构组成，类似于梅氏复合体或胆管板畸形（DPM）。具有DPM模式的肝内胆管癌（iCCA-DPM）是iCCA的一种已确定的变体，而腺纤维瘤样微囊腺癌（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构成一个致瘤谱。对10例手术切除的伴有与AL-TCC相符的BAF（n = 1）或BAF样（n = 9）病变的iCCA进行分析，并与7例iCCA-DPM和26例未明确类型的小胆管iCCA（SD-iCCA）进行比较。AL-TCC队列（6名女性和4名男性）的中位年龄为62岁。肿瘤常为多灶性（70%），平均大小为5.5 cm，常伴有淋巴管侵犯（40%），但无神经侵犯。AL-TCC与iCCA-DPM之间在年龄、性别、肿瘤大小、灶性、淋巴管侵犯、神经侵犯或预后方面无显著差异。当将AL-TCC和iCCA-DPM归为一组（n = 17）并与其他未明确类型的SD-iCCA进行比较时，联合的AL-TCC/iCCA-DPM队列与梅氏复合体、胆管囊肿和/或胆管腺瘤的关联更强（7/17 [41%] 对0/26，P <.001），神经侵犯更少（P =.027），ARID1A缺失更频繁（11/17 [65%] 对3/26 [12%]，P <.001），患者预后更好（P =.036）。Kaplan-Meier分析显示，ARID1A缺失显著改善了患者生存率（P =.046）。总之，伴有BAF或BAF样病变的AL-TCC与iCCA-DPM具有相同的临床病理和组织发生学特征，提示它们相关且可能代表一个不同于其他SD-iCCA的肿瘤发生连续过程。
