Case Report: Epstein-Barr virus negative lymphoepithelioma-like cholangiocarcinoma: a rare tumor that deserves further exploration. Report of a case with distinct genomic and clinical features
Lymphoepithelioma-like cholangiocarcinoma (LEL-CC) is a rare variant of cholangiocarcinoma characterized by undifferentiated malignant epithelial cells and a significant lymphoid infiltrate. Due to its rarity, there is currently no established treatment protocol for LEL-CC, and limited data are avai...
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Frontiers Media S.A.
2025-08-01
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| Series: | Frontiers in Oncology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fonc.2025.1620328/full |
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| author | Salvatore Corallo Salvatore Corallo Chiara Milani Alessandro Vanoli Alessandro Vanoli Anna Gallotti Anna Pagani Francesco Serra Marcello Maestri Valentina Ravetta Angioletta Lasagna Paolo Pedrazzoli Paolo Pedrazzoli Francesco Agustoni Francesco Agustoni |
| author_facet | Salvatore Corallo Salvatore Corallo Chiara Milani Alessandro Vanoli Alessandro Vanoli Anna Gallotti Anna Pagani Francesco Serra Marcello Maestri Valentina Ravetta Angioletta Lasagna Paolo Pedrazzoli Paolo Pedrazzoli Francesco Agustoni Francesco Agustoni |
| author_sort | Salvatore Corallo |
| collection | DOAJ |
| description | Lymphoepithelioma-like cholangiocarcinoma (LEL-CC) is a rare variant of cholangiocarcinoma characterized by undifferentiated malignant epithelial cells and a significant lymphoid infiltrate. Due to its rarity, there is currently no established treatment protocol for LEL-CC, and limited data are available regarding the genomic landscape of this rare tumor. In this report, we present the case of an 84-year-old woman with a history of Hepatitis C-related cirrhosis who was diagnosed with EBV-negative LEL-CC. This tumor presented as a double primary tumor together with a moderately differentiated hepatocellular carcinoma, at the time of first diagnosis. After surgical resection, the patient experienced a relapse with intracolonic metastasis. Comprehensive genomic profiling revealed unique genetic features consistent with LEL carcinoma of other sites of origin. The genetic and clinical characteristics of our case highlight the need for further research on this rare variant of cholangiocarcinoma. Gaining insights into the molecular mechanisms behind this type of cancer could lead to the development of effective targeted therapies or immunotherapeutic approaches. |
| format | Article |
| id | doaj-art-b0c2a8ac92a5438dbacaba58cf3e07e1 |
| institution | Kabale University |
| issn | 2234-943X |
| language | English |
| publishDate | 2025-08-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Oncology |
| spelling | doaj-art-b0c2a8ac92a5438dbacaba58cf3e07e12025-08-20T03:43:02ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2025-08-011510.3389/fonc.2025.16203281620328Case Report: Epstein-Barr virus negative lymphoepithelioma-like cholangiocarcinoma: a rare tumor that deserves further exploration. Report of a case with distinct genomic and clinical featuresSalvatore Corallo0Salvatore Corallo1Chiara Milani2Alessandro Vanoli3Alessandro Vanoli4Anna Gallotti5Anna Pagani6Francesco Serra7Marcello Maestri8Valentina Ravetta9Angioletta Lasagna10Paolo Pedrazzoli11Paolo Pedrazzoli12Francesco Agustoni13Francesco Agustoni14Department of Internal Medicine and Medical Therapy, University of Pavia, Pavia, ItalyUnit of Oncology, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyDepartment of Internal Medicine and Medical Therapy, University of Pavia, Pavia, ItalyDepartment of Molecular Medicine, University of Pavia, Pavia, ItalyUnit of Anatomic Pathology, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyInstitute of Radiology, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyUnit of Oncology, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyUnit of Oncology, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyDivision of General Surgery 1, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyGastroenterology and Endoscopy Unit, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyUnit of Oncology, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyDepartment of Internal Medicine and Medical Therapy, University of Pavia, Pavia, ItalyUnit of Oncology, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyDepartment of Internal Medicine and Medical Therapy, University of Pavia, Pavia, ItalyUnit of Oncology, Fondazione IRCCS Policlinico San Matteo, Pavia, ItalyLymphoepithelioma-like cholangiocarcinoma (LEL-CC) is a rare variant of cholangiocarcinoma characterized by undifferentiated malignant epithelial cells and a significant lymphoid infiltrate. Due to its rarity, there is currently no established treatment protocol for LEL-CC, and limited data are available regarding the genomic landscape of this rare tumor. In this report, we present the case of an 84-year-old woman with a history of Hepatitis C-related cirrhosis who was diagnosed with EBV-negative LEL-CC. This tumor presented as a double primary tumor together with a moderately differentiated hepatocellular carcinoma, at the time of first diagnosis. After surgical resection, the patient experienced a relapse with intracolonic metastasis. Comprehensive genomic profiling revealed unique genetic features consistent with LEL carcinoma of other sites of origin. The genetic and clinical characteristics of our case highlight the need for further research on this rare variant of cholangiocarcinoma. Gaining insights into the molecular mechanisms behind this type of cancer could lead to the development of effective targeted therapies or immunotherapeutic approaches.https://www.frontiersin.org/articles/10.3389/fonc.2025.1620328/fulllymphoepithelioma-like cholangiocarcinomaHCC-CC double primary tumorintracolonic metastasisEBV-negative cholangiocarcinomaHCV-related cirrhosis |
| spellingShingle | Salvatore Corallo Salvatore Corallo Chiara Milani Alessandro Vanoli Alessandro Vanoli Anna Gallotti Anna Pagani Francesco Serra Marcello Maestri Valentina Ravetta Angioletta Lasagna Paolo Pedrazzoli Paolo Pedrazzoli Francesco Agustoni Francesco Agustoni Case Report: Epstein-Barr virus negative lymphoepithelioma-like cholangiocarcinoma: a rare tumor that deserves further exploration. Report of a case with distinct genomic and clinical features Frontiers in Oncology lymphoepithelioma-like cholangiocarcinoma HCC-CC double primary tumor intracolonic metastasis EBV-negative cholangiocarcinoma HCV-related cirrhosis |
| title | Case Report: Epstein-Barr virus negative lymphoepithelioma-like cholangiocarcinoma: a rare tumor that deserves further exploration. Report of a case with distinct genomic and clinical features |
| title_full | Case Report: Epstein-Barr virus negative lymphoepithelioma-like cholangiocarcinoma: a rare tumor that deserves further exploration. Report of a case with distinct genomic and clinical features |
| title_fullStr | Case Report: Epstein-Barr virus negative lymphoepithelioma-like cholangiocarcinoma: a rare tumor that deserves further exploration. Report of a case with distinct genomic and clinical features |
| title_full_unstemmed | Case Report: Epstein-Barr virus negative lymphoepithelioma-like cholangiocarcinoma: a rare tumor that deserves further exploration. Report of a case with distinct genomic and clinical features |
| title_short | Case Report: Epstein-Barr virus negative lymphoepithelioma-like cholangiocarcinoma: a rare tumor that deserves further exploration. Report of a case with distinct genomic and clinical features |
| title_sort | case report epstein barr virus negative lymphoepithelioma like cholangiocarcinoma a rare tumor that deserves further exploration report of a case with distinct genomic and clinical features |
| topic | lymphoepithelioma-like cholangiocarcinoma HCC-CC double primary tumor intracolonic metastasis EBV-negative cholangiocarcinoma HCV-related cirrhosis |
| url | https://www.frontiersin.org/articles/10.3389/fonc.2025.1620328/full |
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