肝内胆管原发性异位肝细胞癌1例并文献复习

Primary Ectopic Hepatocellular Carcinoma in the Intrahepatic Bile Duct: A Case Report and Literature Review

摘要: 目的：探讨肝内胆管异位肝细胞癌(EHCC)的临床病理特征、诊断及治疗策略。方法：回顾性分析1例肝内胆管EHCC患者的临床资料，并复习相关文献。结果：患者因梗阻性黄疸就诊，术前影像学及实验室检查误诊为肝内胆管癌，行右半肝切除 + 胆管重建术后病理证实为EHCC。免疫组化显示HepPar-1、Glypican-3阳性。术后1个月行预防性TACE，随访未见复发。结论：EHCC罕见且易误诊，联合影像学、肿瘤标志物及胆道镜活检有助于术前诊断，本病例通过根治性手术获得了良好的短期效果，与文献报道一致。

Abstract: Objective: To investigate the clinicopathological characteristics, diagnostic approaches, and management of ectopic hepatocellular carcinoma (EHCC) arising in the intrahepatic bile duct. Methods: Clinical data from one patient with EHCC were retrospectively reviewed, along with a literature review. Results: The patient presented with obstructive jaundice and was initially misdiagnosed with intrahepatic cholangiocarcinoma based on imaging and laboratory findings. EHCC was confirmed postoperatively via pathology and immunohistochemistry (HepPar-1+, Glypican-3+) after right hemihepatectomy with bile duct reconstruction. Prophylactic TACE was administered for one month postsurgery, with no recurrence during follow-up. Conclusion: Ectopic hepatocellular carcinoma (EHCC) is rare and prone to misdiagnosis. The combination of imaging, tumor markers, and cholangioscopy-guided biopsy facilitates preoperative diagnosis. This case achieved favorable short-term outcomes through radical surgery, consistent with literature reports.