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, 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 (US), 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 and has a single tumor, resection is the treatment of choice. If both regional lymph node metastasis and multiple tumors are present, drug therapy is the first treatment of choice. If the patient has either regional lymph node metastasis 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.

中文翻译：

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日本肝癌研究组肝内胆管癌临床实践指南

本文介绍了日本肝癌研究组制定的第一版肝内胆管癌 (ICC) 临床实践指南。这些指南包括 1 个治疗算法、5 个背景说明、16 个临床问题和 1 个临床主题，包括病因学、分期、病理学、诊断和治疗。在全球范围内，东亚和东南亚国家的 ICC 发病率很高，日本和西方国家的发病率也逐渐增加。报告的 ICC 危险因素包括肝硬化、乙型/丙型肝炎、饮酒、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，如原发性硬化性胆管炎、肝胆管结石、先天性胆管扩张症和卡罗利病. 化学风险因素包括钍 232、1,2-二氯丙烷和二氯甲烷。CA19-9和CEA被推荐作为ICC早期检测和诊断的肿瘤标志物。腹部超声检查 (US)、CT 和 MRI 是诊断 ICC 的有效成像方式。如果怀疑胆管受侵，检查胆管的影像学方法可能会有用。对于无法切除的病例，当认为有必要进行鉴别诊断和药物治疗选择时，应考虑进行肿瘤活检。Child-Pugh A 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且只有单个肿瘤，则切除是治疗的选择。如果同时存在区域淋巴结转移和多发性肿瘤，药物治疗是首选治疗方法。如果患者有区域淋巴结转移或多发肿瘤，则根据转移范围或肿瘤数量选择切除或药物治疗。如果存在远处转移，药物治疗是首选治疗方法。对于因肝功能储备下降或合并症不适合手术切除或药物治疗的患者，可考虑经皮消融治疗。对于无肝外转移的不可切除ICC，可考虑立体定向放疗（肿瘤大小≤5cm）或粒子放疗（无大小限制）。ICC 一般不适合肝移植，建议对 Child-Pugh C 级肝功能患者进行姑息治疗。如果患者有区域淋巴结转移或多发肿瘤，则根据转移范围或肿瘤数量选择切除或药物治疗。如果存在远处转移，药物治疗是首选治疗方法。对于因肝功能储备下降或合并症不适合手术切除或药物治疗的患者，可考虑经皮消融治疗。对于无肝外转移的不可切除ICC，可考虑立体定向放疗（肿瘤大小≤5cm）或粒子放疗（无大小限制）。ICC 一般不适合肝移植，建议对 Child-Pugh C 级肝功能患者进行姑息治疗。如果患者有区域淋巴结转移或多发肿瘤，则根据转移范围或肿瘤数量选择切除或药物治疗。如果存在远处转移，药物治疗是首选治疗方法。对于因肝功能储备下降或合并症不适合手术切除或药物治疗的患者，可考虑经皮消融治疗。对于无肝外转移的不可切除ICC，可考虑立体定向放疗（肿瘤大小≤5cm）或粒子放疗（无大小限制）。ICC 一般不适合肝移植，建议对 Child-Pugh C 级肝功能患者进行姑息治疗。根据转移程度或肿瘤数目选择切除或药物治疗。如果存在远处转移，药物治疗是首选治疗方法。对于因肝功能储备下降或合并症不适合手术切除或药物治疗的患者，可考虑经皮消融治疗。对于无肝外转移的不可切除ICC，可考虑立体定向放疗（肿瘤大小≤5cm）或粒子放疗（无大小限制）。ICC 一般不适合肝移植，建议对 Child-Pugh C 级肝功能患者进行姑息治疗。根据转移程度或肿瘤数目选择切除或药物治疗。如果存在远处转移，药物治疗是首选治疗方法。对于因肝功能储备下降或合并症不适合手术切除或药物治疗的患者，可考虑经皮消融治疗。对于无肝外转移的不可切除ICC，可考虑立体定向放疗（肿瘤大小≤5cm）或粒子放疗（无大小限制）。ICC 一般不适合肝移植，建议对 Child-Pugh C 级肝功能患者进行姑息治疗。对于因肝功能储备下降或合并症不适合手术切除或药物治疗的患者，可考虑经皮消融治疗。对于无肝外转移的不可切除ICC，可考虑立体定向放疗（肿瘤大小≤5cm）或粒子放疗（无大小限制）。ICC 一般不适合肝移植，建议对 Child-Pugh C 级肝功能患者进行姑息治疗。对于因肝功能储备下降或合并症不适合手术切除或药物治疗的患者，可考虑经皮消融治疗。对于无肝外转移的不可切除ICC，可考虑立体定向放疗（肿瘤大小≤5cm）或粒子放疗（无大小限制）。ICC 一般不适合肝移植，建议对 Child-Pugh C 级肝功能患者进行姑息治疗。