目的:探讨肝胆管囊性肿瘤的临床病理及影像学特点的诊断和治疗。方法:回顾性分析自1990年1月～2007年5月年收集10例经手术病理证实的原发性肝内胆管囊性肿瘤(PCTIHBD),其中囊腺瘤5例和囊腺癌5例的临床病理表现、诊治经验,并结合文献资料加以讨论。结果:全部病例因临床表现和实验室检查缺乏特异性均术前误诊。均行手术并经病理得以证实。其中巨大囊肿采用单纯剥脱术5例,肝叶切除2例,肝部分切除3例。9例术后无胆瘘痊愈;1例巨大左肝囊腺癌术后第6天因肝切面大出血死亡;4例囊腺癌1例于术后13个月肝内复发再次手术切除,另3例分别为半年、4年、5年死于复发。4例囊腺瘤术后5～10年无复发,失访1例。结论:原发性肝内胆管囊性肿瘤,根据临床检查做出确诊很困难,且常合并囊内感染或出血,缺乏特异性临床特征。因此,术前早期诊断主要依靠影像学及术前、术中的病理学检查,即时的综合治疗是很有效的方法。 Objective: To investigate the clinicopathological and imaging diagnosis and treatment of hepatobiliary cystic tumors. Methods: From January 1990 to May 2007, 10 cases of primary intrahepatic cholangiocarcinoma (PCTIHBD) confirmed by surgery and pathology were retrospectively analyzed. Among them, 5 cases of cystadenoma and 5 cases of cystadenocarcinoma Clinical and pathological findings, diagnosis and treatment experience, combined with the literature to be discussed. Results: All cases were preoperatively misdiagnosed due to the lack of specificity of clinical manifestations and laboratory tests. All patients underwent surgery and confirmed by pathology. In which a huge cyst using simple exfoliation in 5 cases, liver resection in 2 cases, partial resection in 3 cases. 9 cases were cured without biliary fistula; 1 case of large left hepatic cystadenocarcinoma died of liver hemorrhage on the 6th day after surgery; 4 cases of cystadenocarcinoma were resected intrahepatic recurrence 13 months after operation, and the other 3 Cases were six months, four years, five years died of recurrence. No recurrence was found in 4 cases of cystadenoma 5 to 10 years after operation, and 1 case was lost to follow-up. Conclusion: Primary intrahepatic cholangiocarcinoma is difficult to diagnose based on clinical examination. It is often associated with intracapsular infection or bleeding and lacks specific clinical features. Therefore, the early preoperative diagnosis depends mainly on imaging and preoperative and postoperative pathological examination, real-time comprehensive treatment is a very effective method.