Epstein - Barr virus - associated lymphoepithelioma - like intrahepatic cholangiocarcinoma: A report of 3 cases and literature review

Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2024 Feb 28;49(2):319-330. doi: 10.11817/j.issn.1672-7347.2024.230298.
[Article in English, Chinese]

Abstract

Hepatic lymphoepithelioma-like carcinoma (LELC) is an extremely rare malignant tumor characterized by undifferentiated malignant epithelial cells and significant lymphatic infiltration. Hepatic LELC mainly includes lymphoepithelioma-like hepatocellular carcinoma (LEL-HCC) and lymphoepithelioma-like intrahepatic cholangiocarcinoma (LEL-CC). Epstein-Barr virus (EBV) infection is considered as an important factor in LELC carcinogenesis. Since 2005, Xiangya Hospital of Central South University has treated a total of 3 patients with EBV-associated LEL-CC, which all showed liver masses by CT scans. After surgical resection, the EBV encoded RNA (EBER) and CK19 expression in all 3 patients were positive, and pathological examination confirmed EBV-associated LEL-CC. Two patients had a good postoperative prognosis, while 1 patient received relevant immunotherapy and chemotherapy after surgery. Based on the analysis of existing literature, the author believes that hepatic LELC can be included in the classification of liver tumors, which will provide new ideas for the accurate diagnosis and treatment of hepatic LELC.

肝淋巴上皮瘤样癌(Lymphoepithelioma-like carcinoma,LELC)是一种极其罕见的恶性肿瘤,其特点是未分化的恶性上皮细胞及明显的淋巴浸润。肝LELC主要包括淋巴上皮瘤样肝细胞癌(lymphoepithelioma-like hepatocellular carcinoma,LEL-HCC)和淋巴上皮瘤样肝内胆管癌(lymphoepithelioma-like intrahepatic cholangiocarcinoma,LEL-CC)。EB病毒(Epstein-Barr virus,EBV)感染被认为是LELC癌变的重要因素。中南大学湘雅医院自2005年以来共收治3例EBV相关LEL-CC患者,CT均提示肝脏肿块,经手术切除后,3例患者EBV编码的RNA(EBV-encoded RNA,EBER)和CK19表达均为阳性,病理学证实为EBV相关的LEL-CC。2例患者术后预后良好,1例患者术后接受相关免疫治疗及化学治疗。结合现有文献分析,笔者认为可将肝LELC纳入肝肿瘤的分类,这将为肝LELC的精准诊断及治疗提供新思路。.

肝淋巴上皮瘤样癌(Lymphoepithelioma-like carcinoma,LELC)是一种极其罕见的恶性肿瘤,其特点是未分化的恶性上皮细胞及明显的淋巴浸润。肝LELC主要包括淋巴上皮瘤样肝细胞癌(lymphoepithelioma-like hepatocellular carcinoma,LEL-HCC)和淋巴上皮瘤样肝内胆管癌(lymphoepithelioma-like intrahepatic cholangiocarcinoma,LEL-CC)。EB病毒(Epstein-Barr virus,EBV)感染被认为是LELC癌变的重要因素。中南大学湘雅医院自2005年以来共收治3例EBV相关LEL-CC患者,CT均提示肝脏肿块,经手术切除后,3例患者EBV编码的RNA(EBV-encoded RNA,EBER)和CK19表达均为阳性,病理学证实为EBV相关的LEL-CC。2例患者术后预后良好,1例患者术后接受相关免疫治疗及化学治疗。结合现有文献分析,笔者认为可将肝LELC纳入肝肿瘤的分类,这将为肝LELC的精准诊断及治疗提供新思路。

Keywords: Epstein-Barr virus; hepatocellular carcinoma; lymphoepithelioma like intrahepatic cholangiocarcinoma; lymphoepithelioma-like carcinoma.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Bile Duct Neoplasms* / pathology
  • Bile Duct Neoplasms* / virology
  • Bile Ducts, Intrahepatic / pathology
  • Cholangiocarcinoma* / pathology
  • Cholangiocarcinoma* / virology
  • Epstein-Barr Virus Infections* / complications
  • Female
  • Herpesvirus 4, Human / genetics
  • Herpesvirus 4, Human / isolation & purification
  • Humans
  • Liver Neoplasms / pathology
  • Liver Neoplasms / virology
  • Male
  • Middle Aged