Epstein-Barr Virus-associated Mixed Lymphoepithelioma-like Carcinoma and Adenocarcinoma of the Gall Bladder: An Unusual Entity

Schmincke described lymphoepithelioma as an undifferentiated carcinoma with abundant lymphoid stroma in the nasopharynx. Tumors with a similar histomorphology in extrapharyngeal areas have been referred to as lymphoepithelioma-like carcinoma (LELC). The association of an Ebstein-Barr virus (EBV) i...

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Main Authors: Niharika Jain, Surbhi Goyal, Puja Sakhuja, Sundeep Saluja
Format: Article
Language:English
Published: Jin Publishing & Printing Co. 2022-09-01
Series:The Korean Journal of Gastroenterology
Subjects:
Online Access:https://www.kjg.or.kr/journal/view.html?uid=5749&vmd=Full
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author Niharika Jain
Surbhi Goyal
Puja Sakhuja
Sundeep Saluja
author_facet Niharika Jain
Surbhi Goyal
Puja Sakhuja
Sundeep Saluja
author_sort Niharika Jain
collection DOAJ
description Schmincke described lymphoepithelioma as an undifferentiated carcinoma with abundant lymphoid stroma in the nasopharynx. Tumors with a similar histomorphology in extrapharyngeal areas have been referred to as lymphoepithelioma-like carcinoma (LELC). The association of an Ebstein-Barr virus (EBV) infection with lymphoepithelioma is well established in the nasopharynx but not so well at the extrapharyngeal sites. Only four cases of LELC have been reported in the gall bladder, of which all were negative for the EBV. This paper reports the first case of an EBV-associated mixed gall bladder carcinoma exhibiting a distinct phenotype of LELC and adenocarcinoma with mucinous differentiation. The EBV was confirmed by the strong granular membranous and cytoplasmic expression of LMP-1 (latent membrane protein-1) on immunohistochemistry and nuclear EBER RNA on chromogen in-situ hybridization in the tumor. This is the first case of LELC positive for EBV in the gall bladder. LELC has a more favorable prognosis than conventional adenocarcinoma or squamous cell carcinoma, irrespective of the site. Although a higher T stage and nodal metastasis were exceptional in the present case in contrast to the previous cases, the EBV-associated lymphocytic response might limit the disease spread and confer better overall survival and prognosis in these patients. Nevertheless, more prospective studies with a larger cohort will be needed to understand the pathogenesis, biological behavior, and prognosis of this rare entity.
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spelling doaj.art-8f27e41b27c443408b5a1bf2e896e3ed2022-12-22T04:27:13ZengJin Publishing & Printing Co.The Korean Journal of Gastroenterology1598-99922233-68692022-09-0178318819410.4166/kjg.2021.045Epstein-Barr Virus-associated Mixed Lymphoepithelioma-like Carcinoma and Adenocarcinoma of the Gall Bladder: An Unusual EntityNiharika Jain0Surbhi Goyal1https://orcid.org/0000-0002-0554-9540Puja Sakhuja2Sundeep Saluja3Departments of Pathology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, IndiaDepartments of Pathology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, IndiaDepartments of Pathology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, IndiaDepartments of Gastrointestinal Surgery, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, IndiaSchmincke described lymphoepithelioma as an undifferentiated carcinoma with abundant lymphoid stroma in the nasopharynx. Tumors with a similar histomorphology in extrapharyngeal areas have been referred to as lymphoepithelioma-like carcinoma (LELC). The association of an Ebstein-Barr virus (EBV) infection with lymphoepithelioma is well established in the nasopharynx but not so well at the extrapharyngeal sites. Only four cases of LELC have been reported in the gall bladder, of which all were negative for the EBV. This paper reports the first case of an EBV-associated mixed gall bladder carcinoma exhibiting a distinct phenotype of LELC and adenocarcinoma with mucinous differentiation. The EBV was confirmed by the strong granular membranous and cytoplasmic expression of LMP-1 (latent membrane protein-1) on immunohistochemistry and nuclear EBER RNA on chromogen in-situ hybridization in the tumor. This is the first case of LELC positive for EBV in the gall bladder. LELC has a more favorable prognosis than conventional adenocarcinoma or squamous cell carcinoma, irrespective of the site. Although a higher T stage and nodal metastasis were exceptional in the present case in contrast to the previous cases, the EBV-associated lymphocytic response might limit the disease spread and confer better overall survival and prognosis in these patients. Nevertheless, more prospective studies with a larger cohort will be needed to understand the pathogenesis, biological behavior, and prognosis of this rare entity.https://www.kjg.or.kr/journal/view.html?uid=5749&vmd=Fullgallbladder neoplasmlymphoepithelioma-like carcinomaepstein-barr virusadenocarcinoma
spellingShingle Niharika Jain
Surbhi Goyal
Puja Sakhuja
Sundeep Saluja
Epstein-Barr Virus-associated Mixed Lymphoepithelioma-like Carcinoma and Adenocarcinoma of the Gall Bladder: An Unusual Entity
The Korean Journal of Gastroenterology
gallbladder neoplasm
lymphoepithelioma-like carcinoma
epstein-barr virus
adenocarcinoma
title Epstein-Barr Virus-associated Mixed Lymphoepithelioma-like Carcinoma and Adenocarcinoma of the Gall Bladder: An Unusual Entity
title_full Epstein-Barr Virus-associated Mixed Lymphoepithelioma-like Carcinoma and Adenocarcinoma of the Gall Bladder: An Unusual Entity
title_fullStr Epstein-Barr Virus-associated Mixed Lymphoepithelioma-like Carcinoma and Adenocarcinoma of the Gall Bladder: An Unusual Entity
title_full_unstemmed Epstein-Barr Virus-associated Mixed Lymphoepithelioma-like Carcinoma and Adenocarcinoma of the Gall Bladder: An Unusual Entity
title_short Epstein-Barr Virus-associated Mixed Lymphoepithelioma-like Carcinoma and Adenocarcinoma of the Gall Bladder: An Unusual Entity
title_sort epstein barr virus associated mixed lymphoepithelioma like carcinoma and adenocarcinoma of the gall bladder an unusual entity
topic gallbladder neoplasm
lymphoepithelioma-like carcinoma
epstein-barr virus
adenocarcinoma
url https://www.kjg.or.kr/journal/view.html?uid=5749&vmd=Full
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AT pujasakhuja epsteinbarrvirusassociatedmixedlymphoepitheliomalikecarcinomaandadenocarcinomaofthegallbladderanunusualentity
AT sundeepsaluja epsteinbarrvirusassociatedmixedlymphoepitheliomalikecarcinomaandadenocarcinomaofthegallbladderanunusualentity