背景:肝门部胆管癌(phCCC)是一种恶性恶性肿瘤。对于不可切除的phCCC患者的最佳治疗尚无共识。本综述旨在收集肝移植和肝切除术作为原发性肝癌治疗的现有证据,并为临床实践提供更好的指导。
方法:搜索在PubMed中进行,Embase,科克伦,和LILACS。手动搜索相关参考文献。纳入标准为:英语或葡萄牙语文献中的报告,a)确诊为phCCC的患者;b)具有治愈意图的患者;c)具有肝切除和肝移植结果的患者。病例报告,reviews,信件,社论,全文不可用的会议摘要和论文被排除在分析之外。
结果:目前的大多数文献都是基于观察性回顾性研究,证据等级较低。与全身化疗或姑息治疗相比,肝切除术具有更好的长期预后,对于某些不可切除的原发性肝癌患者,肝移植是一种很好的替代方法。所有切除或移植的候选人都应在医学上适合并且没有肝内或肝外疾病。作为一般规则,患者表现为纵向尺寸>3厘米或延伸到胆囊管以下的肿瘤,淋巴结疾病,证实肝外播散;术中诊断为转移性疾病;过去五年内其他恶性肿瘤病史,并且未完成放化疗方案,医学上不适合移植,不应考虑.其中一些标准应单独评估。肝移植或切除应仅在经验丰富的肝胆中心考虑。任何决策都必须基于多学科评估。
结论:phCCC是一种复杂的疾病,发病率很高。手术疗法,包括肝切除和肝移植,是更好的长期无病生存的最佳选择。
BACKGROUND: Perihilar cholangiocarcinoma (phCCC) is a dismal malignancy. There is no consensus regarding the best treatment for patients with unresectable phCCC. The present
review aimed to gather the current pieces of evidence for liver transplantation and liver resection as a treatment for phCCC and to build better guidance for clinical practice.
METHODS: The search was conducted in PubMed, Embase, Cochrane, and LILACS. The related references were searched manually. Inclusion criteria were: reports in English or Portuguese literature that a) patients with confirmed diagnosis of phCCC; b) patients treated with a curative intent; c) patients with the outcomes of liver resection and liver transplantation. Case reports, reviews, letters, editorials, conference abstracts and papers with full-text unavailability were excluded from the analysis.
RESULTS: Most of the current literature is based on observational retrospective studies with low grades of evidence. Liver resection has better long-term outcomes than systemic chemotherapy or palliation therapy and liver transplantation is a good alternative for selected patients with unresectable phCCC. All candidates for resection or transplantation should be medically fit and free of intrahepatic or extrahepatic diseases. As a general rule, patients presenting with a tumor having a longitudinal size > 3 cm or extending below the cystic duct, lymph node disease, confirmed extrahepatic dissemination; intraoperatively diagnosed metastatic disease; a history of other malignancies within the last five years, and did not complete chemoradiation regimen and were medically unfit should not be considered for transplantation. Some of these criteria should be individually assessed. Liver transplantation or resection should only be considered in highly experienced hepatobiliary centers, and any decision-making must be based on a multidisciplinary evaluation.
CONCLUSIONS: phCCC is a complex condition with high morbidity. Surgical therapies, including hepatectomy and liver transplantation, are the best option for better long-term disease-free survival.