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Co-Occurrence of EBV-Positive Mucocutaneous Ulcer (EBV-MCU) and CLL/SLL in the Head and Neck Region

EBV-positive mucocutaneous ulcer (EBV-MCU) was classified as a rare new entity of the lymphoproliferative B-cell diseases by the WHO in 2017 and must be distinguished from head and neck squamous cell carcinoma by early biopsy. The aim of the study is to raise awareness of the disease and to give a r...

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Autores principales: Bott, Patricia, Oschlies, Ilske, Radeloff, Andreas, Loewenthal, Maureen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9026795/
https://www.ncbi.nlm.nih.gov/pubmed/35448198
http://dx.doi.org/10.3390/curroncol29040224
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author Bott, Patricia
Oschlies, Ilske
Radeloff, Andreas
Loewenthal, Maureen
author_facet Bott, Patricia
Oschlies, Ilske
Radeloff, Andreas
Loewenthal, Maureen
author_sort Bott, Patricia
collection PubMed
description EBV-positive mucocutaneous ulcer (EBV-MCU) was classified as a rare new entity of the lymphoproliferative B-cell diseases by the WHO in 2017 and must be distinguished from head and neck squamous cell carcinoma by early biopsy. The aim of the study is to raise awareness of the disease and to give a review of the current literature and a recommendation for EBV-MCU management. All EBV-MCU cases of the head and neck region published so far were included. We also report a case of a pharyngeal EBV-MCU in an 89-year-old patient who was immunosuppressed by chronic lymphatic leukaemia/small lymphocytic lymphoma (CLL/SLL). In contrast to all previously described cases, histopathology showed a co-infiltration of EBV-MCU and CLL/SLL. A total of 181 cases were identified on PubMed and summarised. EBV-MCU was predominantly caused by immunosuppressive drug therapy. Complete remission could be achieved in 68% of cases and was mainly attributed to a reduction of the immunosuppressive therapy alone (72%). However, some severe cases require more aggressive treatment. Regarding the various histopathologic similarities to other lymphoproliferative disorders, the diagnosis of EBV-MCU can be misleading, with a great impact on patient care and treatment. This diagnosis must be made with caution and requires a combination of clinical, morphological and immunophenotypic features.
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spelling pubmed-90267952022-04-23 Co-Occurrence of EBV-Positive Mucocutaneous Ulcer (EBV-MCU) and CLL/SLL in the Head and Neck Region Bott, Patricia Oschlies, Ilske Radeloff, Andreas Loewenthal, Maureen Curr Oncol Review EBV-positive mucocutaneous ulcer (EBV-MCU) was classified as a rare new entity of the lymphoproliferative B-cell diseases by the WHO in 2017 and must be distinguished from head and neck squamous cell carcinoma by early biopsy. The aim of the study is to raise awareness of the disease and to give a review of the current literature and a recommendation for EBV-MCU management. All EBV-MCU cases of the head and neck region published so far were included. We also report a case of a pharyngeal EBV-MCU in an 89-year-old patient who was immunosuppressed by chronic lymphatic leukaemia/small lymphocytic lymphoma (CLL/SLL). In contrast to all previously described cases, histopathology showed a co-infiltration of EBV-MCU and CLL/SLL. A total of 181 cases were identified on PubMed and summarised. EBV-MCU was predominantly caused by immunosuppressive drug therapy. Complete remission could be achieved in 68% of cases and was mainly attributed to a reduction of the immunosuppressive therapy alone (72%). However, some severe cases require more aggressive treatment. Regarding the various histopathologic similarities to other lymphoproliferative disorders, the diagnosis of EBV-MCU can be misleading, with a great impact on patient care and treatment. This diagnosis must be made with caution and requires a combination of clinical, morphological and immunophenotypic features. MDPI 2022-04-15 /pmc/articles/PMC9026795/ /pubmed/35448198 http://dx.doi.org/10.3390/curroncol29040224 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Bott, Patricia
Oschlies, Ilske
Radeloff, Andreas
Loewenthal, Maureen
Co-Occurrence of EBV-Positive Mucocutaneous Ulcer (EBV-MCU) and CLL/SLL in the Head and Neck Region
title Co-Occurrence of EBV-Positive Mucocutaneous Ulcer (EBV-MCU) and CLL/SLL in the Head and Neck Region
title_full Co-Occurrence of EBV-Positive Mucocutaneous Ulcer (EBV-MCU) and CLL/SLL in the Head and Neck Region
title_fullStr Co-Occurrence of EBV-Positive Mucocutaneous Ulcer (EBV-MCU) and CLL/SLL in the Head and Neck Region
title_full_unstemmed Co-Occurrence of EBV-Positive Mucocutaneous Ulcer (EBV-MCU) and CLL/SLL in the Head and Neck Region
title_short Co-Occurrence of EBV-Positive Mucocutaneous Ulcer (EBV-MCU) and CLL/SLL in the Head and Neck Region
title_sort co-occurrence of ebv-positive mucocutaneous ulcer (ebv-mcu) and cll/sll in the head and neck region
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9026795/
https://www.ncbi.nlm.nih.gov/pubmed/35448198
http://dx.doi.org/10.3390/curroncol29040224
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