Rouzbahman Marjan, Serra Stefano, Adsay N Volkan, Bejarano Pablo A, Nakanuma Yasuni, Chetty Runjan
Department of Pathology, University Health Network/University of Toronto, Toronto, Canada.
Pathology. 2007 Aug;39(4):413-8. doi: 10.1080/00313020701444531.
Oncocytic change in papillary neoplasms of the biliary tract is a very uncommon finding with little known about pathogenesis, immunophenotype and prognosis, especially in comparison to similar lesions in the pancreatic ductal system. We report four cases of oncocytic biliary intraductal papillary neoplasms (IPNs), highlighting the clinicopathological characteristics of these tumours, the immunohistochemical profile with regard to Wnt pathway proteins and mucin core protein (MUC) status, and compare these findings with the oncocytic variant of intraductal papillary mucinous neoplasm (IPMN) of the pancreas.
Four cases of oncocytic IPN of the extrapancreatic, biliary tree (two with accompanying invasive carcinomas) were examined for mucin profiles and Wnt signalling proteins. The cases were stained for: beta-catenin, c-myc, glutathione synthase kinase (GSK), E-cadherin, cyclin D1, and adenomatous polyposis coli (APC), and MUC1, MUC2, MUC3, MUC4, MUC5AC, MUC5B and MUC6, using standard immunohistochemistry.
The cases occurred in three males and one female, ranging in age from 59 to 81 years. The lesions caused obstructive symptoms related to the biliary tree as well as non-specific abdominal symptoms. Typically, cystic lesions were noted grossly. All four of the IPNs were composed of distinctive oncocytic cells. The invasive carcinomas accompanying two of the cases were also composed of oncocytes. None of the cases showed aberrant expression of the Wnt signalling proteins, although cyclin D1 was markedly over-expressed in all four cases. Three of four cases showed the following mucin profile: MUC3, MUC4, MUC5AC, MUC5B and MUC6 positive.
The Wnt pathway proteins (especially beta-catenin and E-cadherin) are expressed normally in oncocytic variants of intraductal papillary neoplasms of the biliary tree, and the mucin profile is similar to their counterparts in the pancreas.
胆管乳头状肿瘤中的嗜酸性细胞化生是一种非常罕见的现象，其发病机制、免疫表型及预后鲜为人知，尤其是与胰腺导管系统中的类似病变相比。我们报告4例嗜酸性胆管内乳头状肿瘤（IPN），重点阐述这些肿瘤的临床病理特征、Wnt信号通路蛋白的免疫组化特征及黏蛋白核心蛋白（MUC）状态，并将这些结果与胰腺导管内乳头状黏液性肿瘤（IPMN）的嗜酸性细胞变体进行比较。
对4例胰腺外胆管树的嗜酸性IPN（2例伴有浸润性癌）进行黏液谱和Wnt信号蛋白检测。采用标准免疫组化方法对病例进行β-连环蛋白、c-myc、谷胱甘肽合成酶激酶（GSK）、E-钙黏蛋白、细胞周期蛋白D1和腺瘤性息肉病蛋白（APC）以及MUC1、MUC2、MUC3、MUC4、MUC5AC、MUC5B和MUC6染色。
病例包括3名男性和1名女性，年龄在59至81岁之间。病变引起与胆管树相关的梗阻症状以及非特异性腹部症状。大体上通常可见囊性病变。所有4例IPN均由独特的嗜酸性细胞组成。其中2例伴有的浸润性癌也由嗜酸性细胞组成。尽管所有4例病例中细胞周期蛋白D1均明显过表达，但无一例显示Wnt信号蛋白的异常表达。4例中有3例显示以下黏液谱：MUC3、MUC4、MUC5AC、MUC5B和MUC6阳性。
Wnt信号通路蛋白（尤其是β-连环蛋白和E-钙黏蛋白）在胆管内乳头状肿瘤的嗜酸性细胞变体中正常表达，且黏液谱与胰腺中的对应物相似。
