Andraus Wellington, Tustumi Francisco, Santana Alexandre Chagas, Pinheiro Rafael Soares Nunes, Waisberg Daniel Reis, Lopes Liliana Ducatti, Arantes Rubens Macedo, Santos Vinicius Rocha, de Martino Rodrigo Bronze, D'Albuquerque Luiz Augusto Carneiro
Department of Gastroenterology, Transplantation Unit, Universidade de São Paulo, São Paulo, Brazil.
Department of Gastroenterology, Transplantation Unit, Universidade de São Paulo, São Paulo, Brazil.
Hepatobiliary Pancreat Dis Int. 2024 Apr;23(2):139-145. doi: 10.1016/j.hbpd.2024.01.003. Epub 2024 Jan 20.
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.
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.
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.
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.
肝门部胆管癌（phCCC）是一种预后不良的恶性肿瘤。对于不可切除的phCCC患者的最佳治疗方法尚无共识。本综述旨在收集目前关于肝移植和肝切除治疗phCCC的证据，并为临床实践建立更好的指导。
检索了PubMed、Embase、Cochrane和LILACS。手动检索相关参考文献。纳入标准为：英文或葡萄牙语文献报道中，a）确诊为phCCC的患者；b）接受根治性治疗的患者；c）有肝切除和肝移植结果的患者。分析排除病例报告、综述、信件、社论、会议摘要以及无全文的论文。
目前大多数文献基于证据等级较低的观察性回顾性研究。肝切除的长期疗效优于全身化疗或姑息治疗，肝移植是部分不可切除phCCC患者的良好选择。所有切除或移植的候选者应身体健康，无肝内或肝外疾病。一般来说，肿瘤纵向尺寸>3 cm或延伸至胆囊管以下、有淋巴结疾病、确诊有肝外转移、术中诊断为转移性疾病、过去五年内有其他恶性肿瘤病史、未完成放化疗方案且身体状况不佳的患者不应考虑移植。其中一些标准应个别评估。肝移植或切除仅应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。
phCCC是一种发病率高的复杂疾病。包括肝切除术和肝移植在内的手术治疗是实现更好的长期无病生存的最佳选择。
