摘要目的：探讨胰腺导管内乳头状黏液性肿瘤（ IPMN）的临床病理学特征、免疫组织化学及预后等特点。方法分析61例IPMN的临床特点，对标本进行病理形态学观察、免疫组织化学及预后分析。结果61例IPMN中有33例肿瘤发生在胰头部，14例发生在胰体尾部，14例位于胰腺全部；平均发病年龄为61.8岁。37例首发症状为腹部不适，14例为查体发现。61例影像学均发现胰管扩张或胰腺囊实性肿物。大体上，32例为多房囊肿，囊内含黏液并见乳头状肿物，胰腺导管扩张，13例为囊实性结节。显微镜下，胃型16例、肠型21例、胰胆管型21例、嗜酸细胞型3例；IPMN伴轻度异型增生有13例、伴中度异型增生有13例、伴重度异型增生有6例、IPMN相关浸润性癌29例。浸润性癌以胰腺导管腺癌为主（23／29，79.3％），其次是胶样癌（4／29，13.8％），其他类型少见（2／29，6.9％）。免疫组织化学染色显示 MUC5AC、MUC2、MUC1阳性率分别为89.4％（51／57）、36.8％（21／57）和28.3％（13／46），嗜酸细胞型MUC1仅1例阳性。61例IPMN中6例失访，55例患者随访时间12～112个月，中位随访时间为32个月，平均随访时间36个月，5年生存率为76％；IPMN不伴有浸润性癌的患者5年生存率为100％，3例复发；IPMN伴有浸润性癌的患者3年存活率为55％，12／13死于术后2年内。结论 IPMN是最常见的胰腺导管内病变，多表现为胰腺囊肿。病理分型及分类有明确的诊断标准。免疫组织化学对诊断和分型具有一定的帮助；MUC5AC多阳性表达，MUC1为胰胆管型IPMN标志物，MUC2为肠型IPMN的标志物。 IPMN伴轻度、中度及重度异型增生的患者手术切除后预后良好，IPMN相关浸润性癌以导管腺癌为主，患者预后较差，手术后2年内病死率高；复发对生存期影响不大。

abstractsObjective To study the clinicopathologic and immunohistochemical features, and the prognosis of intraductal papillary mucinous neoplasms ( IPMN ) of the pancreas.Methods The clinical findings, morphologic features, immunophenotype and prognosis were investigated in 61 cases of IPMN.Results Of these 61 cases, 33 were in the pancreatic head and 14 were in the body and tail, and 14 in the entire pancreas.The average patients′age was 61.8 years.The initial symptom was abdominal pain in 37 cases, and the tumors were detected at routine checkup in 14 cases.The imaging examination showed dilated ducts and/or cystic and solid masses.Grossly, 32 cases were multi-loculated cystic masses containing mucin and papillary areas;13 cases were solid.Microscopically, the IPMN showed four patterns, including gastric-type ( 16 cases ) , intestinal-type ( 21 cases ) , pancreatobiliary-type ( 21 cases ) and eosinophilic-type (3 cases).The IPMN cohort included 13, 13 and 6 IPMN with low, intermediate and high-grade dysplasia respectively, and 29 IPMN associated with invasive carcinoma.The IPMN associated carcinomas were mainly ductal adenocarcinoma ( 23/29, 79.3%), followed by colloid carcinoma ( 4/29, 13.8%) and undifferentiated carcinoma (2/29,6.9%).Immunohistochemically, IPMN expressed MUC5AC (51/57, 89.4%), MUC2 (21/57,36.8%), and MUC1 (13/46,28.3%).The mean postoperative follow-up period was 32 months ( range 12-112 months).Six of 61 patients were lost to follow-up.Overall 5-year survival rate was 76%.The 5-year survival rate of IPMN with low, intermediate or high-grade dysplasia was 100%, and recurrence was local in 3 patients.The 3-year survival rate of IPMN associated with invasive carcinoma was 55%.12 of 13 patients died within 2 years after operation.Conclusions IPMN is a common cystic neoplasm of the pancreas located in the ducts.The pathologic types and classifications are clearly defined.MUC stains are helpful for the diagnosis and papillary typing.IPMN with invasive carcinoma was associated with significantly worse survival than IPMN with dysplasia.