Clinicopathological features of a rare cancer: Intrahepatic lymphoepithelioma-like cholangiocarcinoma with Epstein-Barr virus infection.
Humans
Female
Adult
Middle Aged
Aged
Male
Epstein-Barr Virus Infections
/ complications
Herpesvirus 4, Human
/ genetics
Retrospective Studies
Cholangiocarcinoma
/ pathology
Carcinoma, Squamous Cell
Prognosis
Bile Ducts, Intrahepatic
/ pathology
Bile Duct Neoplasms
/ therapy
Chromosomal Proteins, Non-Histone
Clinicopathological feature
Epstein-Barr virus
Lymphoepithelioma-like cholangiocarcinoma
Prognosis
Treatment
Journal
Clinics and research in hepatology and gastroenterology
ISSN: 2210-741X
Titre abrégé: Clin Res Hepatol Gastroenterol
Pays: France
ID NLM: 101553659
Informations de publication
Date de publication:
Dec 2023
Dec 2023
Historique:
received:
06
09
2023
revised:
15
10
2023
accepted:
06
11
2023
medline:
6
12
2023
pubmed:
10
11
2023
entrez:
9
11
2023
Statut:
ppublish
Résumé
Epstein-Barr virus-related lymphoepithelioma-like cholangiocarcinoma (EBV-LELCC), a subtype of intrahepatic cholangiocarcinoma (IHCC), is an extremely rare cancer. To date, only few cases have been reported. Therefore, more studies are needed to provide new insights into its clinicopathological characteristics and treatment. We retrospectively collected data from 16 EBV-LELCC patients admitted to our hospital between January 2013 and February 2022. We summarized their clinical characteristics and analyzed the genomic features of 5 patients by whole-exon sequencing. In addition, the Kaplan-Meier method was used to assess the prognostic differences between EBV-LELCC and EBV-negative IHCC. A total of 16 EBV-LELCC patients aged between 35 and 70 were included in this study and were characterized by female predominance. Eight genetic mutations including KMT2C, ARID1B, BAZ1A, NPM1, POLE, PER3, TOPBP1, USP1 were identified from 5 patients. There were 11 stage I, 2 stage III and 3 stage IV patients in this study. The overall survival of stage I and stage III EBV-LELCC patients after radical surgery was significantly better than that of EBV-negative IHCC patients with matched stage (p = 0.0119). Notably, a stage IV patient treated with a variety of antitumor modalities including surgery, interventional therapy, radiotherapy, chemotherapy, targeted therapy and immunotherapy achieved long-term survival of more than seven years. Altogether, EBV-LELCC presents a more favorable prognosis than IHCC. This study suggests that patients with early EBV-LELCC have a good prognosis after radical surgery, and even patients with advanced EBV-LELCC are expected to have a longer survival under appropriate and timely treatment. For such a rare cancer with unique clinicopathological features and molecular patterns, more research is needed to facilitate its diagnosis and treatment.
Identifiants
pubmed: 37944749
pii: S2210-7401(23)00169-9
doi: 10.1016/j.clinre.2023.102244
pii:
doi:
Substances chimiques
BAZ1A protein, human
0
Chromosomal Proteins, Non-Histone
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
102244Informations de copyright
Copyright © 2023. Published by Elsevier Masson SAS.
Déclaration de conflit d'intérêts
Declaration of Competing Interest The authors declare that they have no conflict of interest.