摘要目的：总结肝内胆管内乳头状黏液性肿瘤的临床病理学及免疫组织化学特点。方法搜集并分析北京协和医院病理科诊断的25例发生在肝内胆管内乳头状黏液性肿瘤患者的临床表现、肿瘤病理形态特征。结果25例患者，男12例，女13例，年龄26～73岁，平均60．6岁。腹部症状（15／25）为最常见的主诉。多发生在肝左叶（18／25），其次是肝门部（6／25）；肝右叶病变少见（1／25）。影像学检查显示肝内囊性或囊实性占位11例；肝内胆管内占位，伴胆管扩张8例；肝内外胆管明显扩张，可伴有胆道结石，影像检查未见肿物4例；肝内实性占位，伴有肝内外胆管扩张2例。大体观察与影像学结果相符合。显微镜下，胆管内乳头状肿瘤可分为4型，胃型12例、肠型8例、胰胆管型4例、嗜酸细胞型1例。上皮细胞异型性不等，5例伴有轻－中度异型增生；10例伴有重度异型增生；10例发生浸润，浸润癌1例为胶样癌，9例为胆管腺癌。免疫组织化学结果显示，3例MUC1阳性，均为胰胆管型；8例MUC2阳性，均为肠型；24例MUC5AC阳性，阴性的1例为胰胆管型；10例MUC6阳性，胃型、胰胆管型及嗜酸细胞型均可见。结论肝内胆管内乳头状肿瘤是肝脏少见的癌前病变，可发展为胆管腺癌。影像学表现多样，大部分都伴有胆管扩张；大体特点最常见为多房或单房囊肿伴有囊内乳头；显微镜下分为四种类型，胃型、肠型、胰胆管型及嗜酸细胞型，免疫组织化学MUC系列染色有助于分型。

abstractsObjective To study the clinicopathologic and immunohistochemical features of intraductal papillary neoplasms of the bile duct ( IPNB ) of the intrahepatic bile duct.Methods The clinical findings, morphologic features and immunophenotype of 25 cases of IPNB were investigated. Results Of the 25 cases of IPNB, 18 were in the left lobe, 6 were in the hilar bile duct, and 1 was in the right lobe.The average age was 60.6 ( 26 -73 ) years.The presenting symptom was abdominal pain in 15 cases.Imaging results were variable, including multilobular cysts, mass lesions within the dilated ducts, cholangiolithiasis with dilated bile duct, and solid mass.Gross examinations were concordant with imaging findings.Microscopically, all the IPNB could be divided into four papillary patterns, including 12 gastric type, 8 intestinal type, 4 pancreatobiliary type and 1 oncocytic type.Five IPNB showed mild to moderate atypia, 10 showed high-grade atypia and 10 IPNB were associated with carcinomas.The invasive IPNB were mainly bile duct adenocarcinomas(9 cases),and rarely colloid carcinoma (1 case).Immunohistochemically, IPNB expressed MUC5AC ( 96.0%, 24/25 ) , MUC2 ( 32.0%, 8/25 ) , MUC6 ( 40.0%, 10/25 ) and MUC1 (12.0%, 3/25).The different MUC expression was commonly associated with specific histological subtype.Conclusions IPNBs are rare cystic and premalignant neoplasms of the liver.The imaging features vary, but show a common feature of dilated bile duct.The pathologic types and classifications are similar to intraductal papillary mucinous neoplasm of the pancreas.The MUC stains are helpful in diagnosis and histological subtyping.