Abstract: Focus on the diagnosis and treatment of intrahepatic cholangiocarcinoma        SHEN Feng, WANG Kui. Eastern Hepatobiliary Surgical Hospital，Naval Medical University，Shanghai 200438，China
Corresponding author：SHEN Feng，E- mail：shenfengehbh@
sina.com
Abstract    Intrahepatic cholangiocarcinoma （ICC）is a primary liver cancer with the incidence only second to hepatocellular carcinoma（HCC）.  In recent years， the incidence of ICC has also significantly increased globally. ICC patients usually have asymptomatic features at early disease stage. The diagnosis of ICC depends on serological，imaging and pathological examinations. ICC invading the hilum need to be differentiated from hilar cholangiocarcinoma，and ICC with cirrhosis need to be differentiated from mixed type of primary liver cancer and metastatic liver malignancy. Liver resection is still the only established treatment aiming at care for ICC patients，but only a small number of patients have the chance to receive curative resection. ICC usually presents with the aggressive pathological features such as nodal metastasis and vascular invasion. The preoperative diagnosis and evaluation of ICC are the basis for treatment. Routine lymphadenectomy and the range are still inconclusive. The long-term effect of laparoscopic hepatectomy for ICC is still lacking sufficient evidence. The role of liver transplantation for ICC is still controversial. Due to the high recurrence rate after liver resection，development of effective adjuvant therapy has been highly valued. Systemic chemotherapy and molecular targeted therapy may provide survival benefit for patients with unresectable ICCs. Currently，immunotherapy mainly represented by immune checkpoint inhibitors may provide new insights for the treatment of the disease.

Key words: liver neoplasms, intrahepatic cholangiocarcinoma, surgical treatment, adjuvant therapy, epidemiology, systematic treatment

摘要： 肝内胆管癌（ICC）是发病率仅次于肝细胞癌的原发性肝脏恶性肿瘤。近年来ICC在全球范围内的发病率显著升高。该病发病隐匿，初始时无明显临床症状，诊断依赖于血清学、影像学和或病理学，且中央型或伴肝硬化者还须同肝门部胆管癌、混合型肝癌和转移性肝癌等鉴别。肝切除术目前仍是ICC病人获得长期生存的惟一治疗方式，但仅有少部分能够获得根治性切除机会。ICC容易出现淋巴结转移和血管侵犯，术前准确诊断及评估是精准外科治疗的基础，但术中是否常规行区域性淋巴结清扫以及清扫范围尚无定论。肝移植在ICC中的治疗价值亦存在争议。腹腔镜肝切除术治疗ICC的远期效果尚缺乏强有力的临床证据。由于ICC术后的高复发率，对于高危复发病人的辅助性治疗也备受关注。全身化疗和靶向药物治疗为不可手术的中晚期ICC提供了治疗选择，但疗效仍需更多确切性证据。近来以免疫检查点抑制剂为代表的免疫治疗为ICC的系统性治疗提供了新的治疗思路。

关键词: 肝肿瘤, 肝内胆管癌, 外科治疗, 辅助治疗, 流行病学, 系统治疗