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An update on cholangiocarcinoma associated with primary sclerosing cholangitis

机译:与原发性硬化性胆管炎相关的胆管癌的最新进展

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Purpose of review Primary sclerosing cholangitis (PSC) is being diagnosed with increasing frequency. The most feared complication is the presence or development of cholangiocarcinoma (CCA). The present review summarizes recent data with regards to diagnosis, pathobiology and treatment. Recent findings Several investigations have focused on aspects of the molecular biology of CCA in general; such data should be explored now in the context of PSC-related CCA to yield new diagnostic markers and approaches for therapy. Summary CCA has to be suspected in any new PSC patient presenting with jaundice. Exploration should include carbohydrate antigen 19-9 and two imaging techniques. Endoscopic cholangioscopy might become very rewarding. Important progress has been achieved in liver transplantation by the use of preoperative radio-chemotherapy. Molecular biology points to inflammation-induced cytokines with mutagenic action and to the relevance of extracellular matrix proteins for invasion but also for proliferation. Micro-RNAs prove to be very important in the control of cell proliferation, differentiation and apoptosis. Mutated p53, cyclins, wnt/beta-catenin signaling, proliferation indices, mucins, carbohydrate antigen 19-9, CRP and aneuploidy appear to hold significant potential as predictors of outcome in CCA. It is expected that the further unraveling of these molecular processes will ultimately lead to development of tests allowing early diagnosis and to development of medical approaches to retard tumor formation or recurrence following surgical interventions.
机译:复查目的被诊断为原发性硬化性胆管炎(PSC)的频率增加。最令人担忧的并发症是胆管癌(CCA)的存在或发展。本综述总结了有关诊断,病理生物学和治疗的最新数据。最新发现总的来说,一些研究集中在CCA的分子生物学方面。现在应该在与PSC相关的CCA的背景下探索此类数据,以产生新的诊断标记和治疗方法。总结任何出现黄疸的新PSC患者都必须怀疑CCA。探索应包括碳水化合物抗原19-9和两种成像技术。内窥镜胆管镜检查可能会变得非常有益。通过术前放化疗,在肝移植中已取得重要进展。分子生物学指出具有诱变作用的炎症诱导的细胞因子,以及细胞外基质蛋白对于侵袭和增殖的相关性。事实证明,微RNA在控制细胞增殖,分化和凋亡中非常重要。突变的p53,细胞周期蛋白,wnt /β-catenin信号传导,增殖指数,粘蛋白,碳水化合物抗原19-9,CRP和非整倍性似乎具有显着潜力,可作为CCA结局的预测因子。预期这些分子过程的进一步分解最终将导致开发能够早期诊断的测试方法,并导致开发出在手术干预后延缓肿瘤形成或复发的医学方法。

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