目的

探讨EB病毒相关胆管癌临床病理特征、诊治及预后。

方法

回顾性分析2022年11月中山大学孙逸仙纪念医院收治1例EB病毒相关胆管癌伴多发转移患者临床资料。患者男，74岁，因“上腹部不适1月余”入院，穿刺病理活检符合EB病毒相关胆管癌。行4次TACE及紫杉醇+卡瑞利珠单抗+氟尿嘧啶+吉西他滨方案化疗。患者已签署知情同意书，符合医学伦理学规定。检索万方、知网、PubMed“EB病毒相关胆管癌”相关文献，截止检索日期2023年5月31日。

结果

共纳入21篇文献71例EB病毒相关胆管癌患者，其中男24例，女47例；50岁以上45例，50岁以下26例。位于肝左、右叶分别为28、31例；HBV感染27例，HCV感染1例，EB感染12例；AFP升高2例，CA19-9升高12例；肝硬化2例，远处转移7例。47例单发无远处淋巴结转移；12例肝内转移，部分存在有远处淋巴结转移。EB病毒相关胆管癌的3种病理亚型中，肝内胆管细胞性淋巴上皮瘤样癌（LELCC）为最主要的类型，占比68%（48/71），淋巴上皮瘤样肝内胆管细胞癌（LEL-ICC）占比18%（13/71），淋巴上皮瘤样癌（LELC）占比14%（10/71）。87%（62/71）的患者分布在东南沿海省份，仅有13%（9/71）分布在北京、四川及湖北省。59例接受了手术治疗，PD-1免疫治疗1例，射频消融治疗1例，还有6例未提及具体治疗方案。随访时间1～128个月，59例获得随访，其中36例无瘤生存，10例带瘤生存，1例复发后手术治疗，死于肿瘤12例。本院报告患者目前仍处于带瘤生存状态。

结论

EB病毒相关胆管癌作为一种罕见的胆管癌亚型，具有完全不同的临床病理特征及预后。首次就诊时病变以单发为主，LELCC为最主要病理类型，分布存在明显的地域性，东南沿海多发，预后也较普通胆管细胞癌好。

Objective

To investigate the clinicopathological characteristics，diagnosis，treatment and prognosis of Epstein-Barr （EB） virus-associated cholangiocarcinoma.

Methods

Clinical data of one patient with EB virus-associated cholangiocarcinoma complicated with multiple metastases admitted to Sun Yat-sen Memorial Hospital of Sun Yat-sen University in November 2022 were retrospectively analyzed.The 74-year-old male patient was admitted to our hospital due to "epigastric discomfort for more than one month" and underwent biopsy，confirming the diagnosis of EB virus-associated cholangiocarcinoma.He received 4 cycles of TACE combined with paclitaxel + camrelizumab + fluorouracil + gemcitabine chemotherapy. The informed consents of the patient was obtained and the local ethical committee approval was received. Studies related to "EB virus-associated cholangiocarcinoma" were searched from Wanfang Data，CNKI and PubMed from the inception date of databases until May 31，2023.

Results

A total of 21 articles were included. A total of 71 patients with EB virus-associated cholangiocarcinoma were searched，24 male and 47 female，including 45 cases aged＞ 50 years，and 26 cases aged＜50 years. The lesions were located in the left lobe of 28 patients and in the right lobe of 31 cases. 27 cases were infected with HBV，1 case with HCV and 12 cases with EB virus. AFP levels were increased in 2 cases and CA19-9 levels were elevated in 12 cases. 2 cases were complicated with liver cirrhosis and 7 cases with distant metastases.47 cases had single lesion without distant lymph node metastasis. 12 cases developed intrahepatic metastases，some of them had distant lymph node metastases. Among the three pathological subtypes of EB virus-associated cholangiocarcinoma，intrahepatic lymphoepithelioma-like cholangiocarcinoma（LELCC） is the dominant type，accounting for 68% （48/71），lymphoepithelioma-like hepatocellular carcinomas （LEL-HCC） accounted for 18% （13/71），lymphoepithelioma-like carcinoma （LELC） accounted for 14% （10/71），respectively. Approximately 87% （62/71） of the patients were distributed in the southeast coastal provinces，and only 13% （9/71） were distributed in Beijing，Sichuan and Hubei provinces.59 cases underwent surgical resection，1 case received PD-1 immunotherapy，1 case received radiofrequency ablation，and no specific treatment was described in 6 cases，respectively. Follow-up duration was ranged from 1 to 128 months. 59 patients were followed up. Among them，36 cases achieved tumor-free survival，10 cases survived with tumors，1 case underwent surgery after recurrence，and 12 cases died from tumors，respectively. At present，the patient in this report still survives with tumors.

Conclusions

EB virus-associated cholangiocarcinoma，as a rare subtype of cholangiocarcinoma，has completely different clinicopathological characteristics and prognosis. Upon the first admission，the lesion is mainly single. LELCC is the dominant pathological type. Regional distribution is highly different，with a high prevalence in the southeast coastal region. It yields better prognosis than common cholangiocarcinoma.