本文介绍了日本肝癌研究组建立的肝内胆管癌(ICC)临床实践指南的第一版。这些指南包括1种治疗算法,5个背景陈述,16个临床问题,和1个临床主题,包括病因,分期,病理学,诊断,和治疗。全球范围内,据报道,东亚和东南亚国家的ICC发病率很高,在日本和西方国家,发病率逐渐增加。报告的ICC风险因素包括肝硬化,乙型肝炎/丙型肝炎,酒精消费,糖尿病,肥胖,吸烟,非酒精性脂肪性肝炎,和肝吸虫感染,以及胆道疾病,比如原发性硬化性胆管炎,肝胆管结石,先天性胆管扩张症,和Caroli病.化学危险因素包括钍-232,1,2-二氯丙烷,和二氯甲烷。CA19-9和CEA被推荐作为早期发现和诊断ICC的肿瘤标志物。腹部超声检查,CT,MRI是诊断ICC的有效影像学方法。如果怀疑胆管侵犯,检查胆管的成像方式可能是有用的。在无法切除的情况下,当认为鉴别诊断和药物治疗选择有必要时,应考虑进行肿瘤活检。Child-PughA级或B级肝功能患者的主要治疗方法是手术切除和药物治疗。如果患者没有区域淋巴结转移(LNM)并且有单个肿瘤,切除是治疗的选择。如果同时存在区域性LNM和多发性肿瘤,药物治疗是首选治疗方法。如果患者患有区域性LNM或多种肿瘤,选择切除或药物治疗,取决于转移的程度或肿瘤的数量。如果存在远处转移,药物治疗是首选的治疗方法。对于因肝功能储备减少或合并症而不适合手术切除或药物治疗的患者,可考虑经皮消融治疗。对于没有肝外转移的不可切除的ICC,可考虑进行立体定向放射治疗(肿瘤大小≤5cm)或粒子放射治疗(无大小限制).ICC通常不用于肝移植,对于Child-PughC级肝功能患者,建议姑息治疗。
This paper presents the first version of clinical practice
guidelines for intrahepatic cholangiocarcinoma (ICC) established by the Liver Cancer Study Group of Japan. These
guidelines consist of 1 treatment algorithm, 5 background statements, 16 clinical questions, and 1 clinical topic, including
etiology, staging, pathology, diagnosis, and treatments. Globally, a high incidence of ICC has been reported in East and Southeast Asian countries, and the incidence has been gradually increasing in Japan and also in Western countries. Reported risk factors for ICC include cirrhosis, hepatitis B/C, alcohol consumption, diabetes, obesity, smoking, nonalcoholic steatohepatitis, and liver fluke infestation, as well as biliary diseases, such as primary sclerosing cholangitis, hepatolithiasis, congenital cholangiectasis, and Caroli disease. Chemical risk factors include thorium-232, 1,2-dichloropropane, and dichloromethane. CA19-9 and CEA are recommended as tumor markers for early detection and diagnostic of ICC. Abdominal ultrasonography, CT, and MRI are effective imaging modalities for diagnosing ICC. If bile duct invasion is suspected, imaging modalities for examining the bile ducts may be useful. In unresectable cases, tumor biopsy should be considered when deemed necessary for the differential diagnosis and drug therapy selection. The mainstay of treatment for patients with Child-Pugh class A or B liver function is surgical resection and drug therapy. If the patient has no regional lymph node metastasis (LNM) and has a single tumor, resection is the treatment of choice. If both regional LNM and multiple tumors are present, drug therapy is the first treatment of choice. If the patient has either regional LNM or multiple tumors, resection or drug therapy is selected, depending on the extent of metastasis or the number of tumors. If distant metastasis is present, drug therapy is the treatment of choice. Percutaneous ablation therapy may be considered for patients who are ineligible for surgical resection or drug therapy due to decreased hepatic functional reserve or comorbidities. For unresectable ICC without extrahepatic metastasis, stereotactic radiotherapy (tumor size ≤5 cm) or particle radiotherapy (no size restriction) may be considered. ICC is generally not indicated for liver transplantation, and palliative care is recommended for patients with Child-Pugh class C liver function.