Abstract:

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.

Key words: Bile duct neoplasms, EBV associated cholangiocarcinoma, Neoplasm metastasis, Prognosis