肝内胆管囊腺瘤:单中心连续10例诊疗经验

Intrahepatic biliary cystadenoma: experience with 10 consecutive cases at a single center

目的总结肝内胆管囊腺瘤（IBCA）的诊疗经验,提高临床诊治水平。方法回顾性总结分析我中心治疗的连续10例经病理学确诊的IBCA,分析其流行病学、影像学、实验室检查、病理学和预后特点。结果 10例患者均为女性,平均年龄48.9（16~73）岁。无症状、轻微症状和明显症状的患者分别为4、4、2例。影像学检查均为多房性囊性肿块,平均直径为（13.3±4.9）cm。内部分隔、乳头或结节样增生、钙化的显示率分别为90%,60%,20%。病理检查大体均为多房性肿块,镜下检查囊内壁均被覆立方或柱状上皮,基质类型为卵巢样（50%）或纤维样（50%）。10例患者中完整切除8例,部分切除2例。平均随访55.3（12~164）月,所有患者均生存。完整切除者均无复发;部分切除者均已复发,且其中1例已恶变。结论IBCA是一种少见的肝内囊性交界性肿瘤,好发于中年女性,影像学检查是术前诊断主要依据。应完整切除肿瘤,以期获得良好生存。

Objective To summarize the clinical experience with diagnosis and treatment of intrahepatic biliary cystadenoma（IBCA）.Methods We retrospectively analyzed the data of 10 consecutive IBCA cases treated in our department in light of the characteristics of the epidemiology,radiology,lab tests,pathology and prognostic.Results The patients are all female with an average age of 48.9（16-73） years.The number of asymptomatic,slightly symptomatic and severe symptomatic patients was 4,4 and 2,respectively.Radiological examination showed segmented cystic lesions in all the cases with an average diameter is 13.3±4.9 cm.The incidence of segmentation,papillary or nodular hyperplasia,and calcification within the lesions was 90%,60% and 20%,respectively.Macroscopic examination of the specimen showed compartmentation in the lesions,and microscopically,the lesions all showed lining of cubic or columnar epithelium on the inner wall with ovary-like or fibrous stroma.Complete resection of the tumor was achieved in 8 cases and partial resection was performed in 2 cases.The patients were followed up for a mean of 55.3（12-164） months,and none of the patients with complete tumor resection showed recurrence,while both of the two patients with partial resection had postoperative recurrence.Conclusion IBCA is a rare cystic lesion occurring primarily in middle-aged women.The preoperative diagnosis of this disease relies primarily on radiological evidences,and a complete resection of IBCA may prolong the patient survival.