Waisberg Daniel R, Pinheiro Rafael S, Nacif Lucas S, Rocha-Santos Vinicius, Martino Rodrigo B, Arantes Rubens M, Ducatti Liliana, Lai Quirino, Andraus Wellington, D'Albuquerque Luiz C
Disciplina de Transplante de Figado e Orgaos do Aparelho Digestivo, Departamento de Gastroenterologia, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil.
Transplant Unit, Department of Surgery, University of L'Aquila, San Salvatore Hospital, L'Aquila, Italy.
Transl Gastroenterol Hepatol. 2018 Sep 12;3:60. doi: 10.21037/tgh.2018.08.03. eCollection 2018.
Intrahepatic cholangiocarcinoma (ICC) is the second most prevalent primary liver neoplasm after hepatocellular carcinoma (HCC), corresponding to 10% to 15% of cases. Pathologies that cause chronic biliary inflammation and bile stasis are known predisposing factors for development of ICC. The incidence and cancer-related mortality of ICC is increasing worldwide. Most patients remain asymptomatic until advance stage, commonly presenting with a liver mass incidentally diagnosed. The only potentially curative treatment available for ICC is surgical resection. The prognosis is dismal for unresectable cases. The principle of the surgical approach is a margin negative hepatic resection with preservation of adequate liver remnant. Regional lymphadenectomy is recommended at time of hepatectomy due to the massive impact on outcomes caused by lymph node (LN) metastasis. Multicentric disease, tumor size, margin status and tumor differentiation are also important prognostic factors. Staging laparoscopy is warranted in high-risk patients to avoid unnecessary laparotomy. Exceedingly complex surgical procedures, such as major vascular, extrahepatic bile ducts and visceral resections, hepatectomy and autotransplantation, should be implemented in properly selected patients to achieve negative margins. Neoadjuvant therapy may be used in initially unresectable lesions in order to downstage and allow resection. Despite optimal surgical management, recurrence is frustratingly high. Adjuvant chemotherapy with radiation associated with locoregional treatments should be considered in cases with unfavorable prognostic factors. Selected patients may undergo re-resection of tumor recurrence. Despite the historically poor outcomes of liver transplantation for ICC, highly selected patients with unresectable disease, especially those with adequate response to neoadjuvant therapy, may be offered transplant. In this article, we reviewed the current literature in order to highlight the most recent advances and recommendations for the surgical treatment of this aggressive malignancy.
肝内胆管癌（ICC）是仅次于肝细胞癌（HCC）的第二大常见原发性肝脏肿瘤，占病例的10%至15%。导致慢性胆管炎症和胆汁淤积的病理状况是已知的ICC发生的易感因素。ICC的发病率和癌症相关死亡率在全球范围内都在上升。大多数患者在疾病晚期之前仍无症状，通常因偶然诊断出肝脏肿块而就诊。ICC唯一可能治愈的治疗方法是手术切除。对于无法切除的病例，预后很差。手术方法的原则是进行切缘阴性的肝切除并保留足够的肝残余量。由于淋巴结（LN）转移对预后有巨大影响，因此建议在肝切除时进行区域淋巴结清扫。多中心病变、肿瘤大小、切缘状态和肿瘤分化也是重要的预后因素。对于高危患者，分期腹腔镜检查是必要的，以避免不必要的剖腹手术。对于经过适当选择的患者，可以实施极其复杂的手术，如主要血管、肝外胆管和内脏切除、肝切除和自体移植，以实现切缘阴性。新辅助治疗可用于初始无法切除的病变，以便降期并实现切除。尽管进行了最佳的手术管理，但复发率仍然高得令人沮丧。对于具有不良预后因素的病例，应考虑辅助化疗联合放疗及局部治疗。部分患者可能需要对肿瘤复发进行再次切除。尽管ICC肝移植的历史疗效不佳，但对于经过高度选择的无法切除的患者，尤其是那些对新辅助治疗有充分反应的患者，可以考虑进行移植。在本文中，我们回顾了当前的文献，以突出这种侵袭性恶性肿瘤外科治疗的最新进展和建议。
