肝内胆管乳头状瘤病的临床病理特征和诊治分析：附九例报告

Intrahepatic biliary papillomatosis：a report of 9 cases

目的 总结肝内胆管乳头状瘤病的临床病理特点及诊治结果 ,以提高对本病的认识.方法 收集2006年8月至2008年10月经病理证实的9例肝内胆管乳头状瘤病的临床资料,分析其临床表现,影像学发现,病理特征及治疗结果 .结果 本组9例中男2例,女7例,中位年龄59岁,主要临床表现为反复发热、右上腹痛及黄疸等胆管炎征象,8例合并胆道结石或既往有胆道结石手术史,CEA和CA19-9大多正常.B超和CT/MRCP发现有病变部位肝内胆管局限性囊状扩张和肝萎缩,黏液分泌者胆总管扩张尤其明显.全部病例接受根治性手术切除.9例病变均位于左叶,其中7例为黏液分泌型.剖检标本肝内胆管呈囊状改变,病变多发,分布在肝内各级胆管,呈乳头状隆起,直径在2～5 mm,很少超过10 mm,质脆易出血,镜下见不同程度的异型增生和上皮瘤变,其中2例有恶变.术后随访至今,均无复发.结论 本组资料表明肝内胆管乳头状瘤病好发于有胆道结石病史的中老年女性.临床表现为反复发作的胆管炎,影像学表现为肝内外胆管的显著扩张,尤以病变部位为甚,MRCP和ERCP对本病诊断价值较大.临床和病理显示本病是具有很高恶变潜能的交界性肿瘤,根治性手术切除预后良好.术中胆道镜检查和切缘的快速切片有助于提高本病的根治切除率.

Objective Intrahepatic biliary papillomatosis（IBP）is a rare disease that is characterized by multiple numerous papillary adenomas in the intrahepatic biliary duct（IBD）.The clinical features and outcome,however,are not well known.The clinicopathologic features,treatments and follow-up results were retrospectively analyzed in order to improve the efficiency of diagnosis and treatment for the disease.Methods Between August 2006 and October 2008,9 patients were diagnosed with IBP by histological findings at a tertiary referral center,Ningbo Medical Center（University of Ningbo,College of Medicine,Ningbo,China）.The authors retrospectively reviewed the medical records to obtain clinical,radiological and pathologic data.The therapeutic results and follow-up data were also reviewed.Results The ratio of male to female was 2：7 and the middle age was 59 years.Repeated episodes of fever and right upper quadrant abdominal pain with or without jaundice were the common clinical manifestations.There were intrahepatic choledocholithiasis and/or history of previous biliary operation in 8 cases.The level of CA19-9 and CEA were almost normal.Imaging workup demonstrated cyst-like dilatation of intrahepatic biliary tree with or without liver atrophy,which were more obvious in the mucin-hypersecreting IBP.All of the 9 cases underwent curative resection with an adequate resection margin.Macroscopic findings demonstrated that the tumors of 9 cases were all located in the left lobe with mucin-hypersecreting type in 7 cases.The diameter of numerous papillary granular was usually less than 10 mm（from 2 to 5 mm.frequently）and friable,that filled the dilated IHD dispersive.Fine fibrovascular cores lined by dysplastic epithelial cells were frequently found under microscope.Few foci of stromalinvasion were noticed in the two cases with malignant transformation.All of the 9 cases survived and there was no recurrence.Conclusion IBP occurs more often in middle and old women who have history of biliary disease. Repeated episodes of cholangitis are the common clinical manifestations. Extra- and intrahepatic biliary tract dilatation is the common imaging finding.MRCP/ERCP is more valuable than others in diagnosis. Clinical and histological finding shows that IBP is a premalignant disease with high malignant potential. Curative resection should be done as earlyas possible for the long-term survival rate. The use of cholangioscopy in operation and rapid biopsy of resection margin will benefit the curative resection.