Colangelo Matteo, Di Martino Marcello, Polidoro Michela Anna, Forti Laura, Tober Nastassja, Gennari Alessandra, Pagano Nico, Donadon Matteo
Department of Health Sciences, University of Piemonte Orientale, Novara, Italy.
Division of Surgery, University Maggiore Hospital della Carità, Novara, Italy.
Gastroenterol Rep (Oxf). 2025 Jan 26;13:goaf005. doi: 10.1093/gastro/goaf005. eCollection 2025.
Intrahepatic cholangiocarcinoma (iCCA) is an aggressive liver malignancy that arises from second-order biliary epithelial cells. Its incidence is gradually increasing worldwide. Well-known risk factors have been described, although in many cases, they are not identifiable. Treatment options are continuously expanding, but the prognosis of iCCA remains dismal. R0 liver resection remains the only curative treatment, but only a limited number of patients can benefit from it. Frequently, major hepatectomies are needed to completely remove the tumour. This could contraindicate surgery or increase postoperative morbidity in patients with chronic liver disease and small remnant liver volume. In cases of anticipated inadequate future liver remnant, regenerative techniques may be used to expand resectability. The role and extent of lymphadenectomy in iCCA are still matters of debate. Improvements in iCCA diagnosis and better understanding of genetic profiles might lead to optimized surgical approaches and drug therapies. The role of neoadjuvant and adjuvant therapies is broadening, gaining more and more acceptance in clinical practice. Combining surgery with locoregional therapies and novel drugs, such as checkpoint-inhibitors and molecular-targeted molecules, might improve treatment options and survival rates. Liver transplantation, after very poor initial results, is now receiving attention for the treatment of patients with unresectable very early iCCA (i.e. <2 cm) in cirrhotic livers, showing survival outcomes comparable to those of hepatocellular carcinoma. Ongoing prospective protocols are testing the efficacy of liver transplantation for patients with unresectable, advanced tumours confined to the liver, with sustained response to neoadjuvant treatment. In such a continuously changing landscape, the aim of our work is to review the state-of-the-art in the surgical and medical treatment of iCCA.
肝内胆管癌（iCCA）是一种侵袭性肝脏恶性肿瘤，起源于二级胆管上皮细胞。其发病率在全球范围内正逐渐上升。尽管在许多情况下无法明确，但已知的风险因素已被描述。治疗选择在不断扩展，但iCCA的预后仍然不佳。R0肝切除术仍然是唯一的根治性治疗方法，但只有少数患者能从中受益。通常，需要进行大范围肝切除术才能完全切除肿瘤。这可能会使患有慢性肝病且残余肝体积小的患者无法进行手术或增加术后发病率。在预计未来肝残余量不足的情况下，可采用再生技术来扩大可切除性。iCCA中淋巴结清扫的作用和范围仍存在争议。iCCA诊断的改善以及对基因谱的更好理解可能会带来优化的手术方法和药物治疗。新辅助治疗和辅助治疗的作用正在扩大，在临床实践中越来越被接受。将手术与局部区域治疗及新型药物（如检查点抑制剂和分子靶向分子）相结合，可能会改善治疗选择和生存率。肝移植在最初结果非常差之后，现在正受到关注，用于治疗肝硬化肝脏中不可切除的极早期iCCA（即<2厘米）患者，其生存结果与肝细胞癌相当。正在进行的前瞻性方案正在测试肝移植对局限于肝脏的不可切除晚期肿瘤患者的疗效，这些患者对新辅助治疗有持续反应。在这样一个不断变化的形势下，我们工作的目的是回顾iCCA手术和药物治疗的最新进展。
