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Primary oral manifestation of Langerhans cell histiocytosis refractory to conventional therapy but susceptible to BRAF-specific treatment: a case report and review of the literature

Langerhans cell histiocytosis (LCH) is a diagnostic and therapeutic challenge. We report on a rare case of its primary oral manifestation that was treated successfully with the BRAF-specific agent, vemurafenib, after insufficient standard LCH treatment. This case underlines the importance of proper...

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Autores principales: Neckel, Norbert, Lissat, Andrej, von Stackelberg, Arendt, Thieme, Nadine, Doueiri, Mohemed-Salim, Spors, Birgit, Beck-Broichsitter, Benedicta, Heiland, Max, Raguse, Jan-Dirk
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6801882/
https://www.ncbi.nlm.nih.gov/pubmed/31666812
http://dx.doi.org/10.1177/1758835919878013
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author Neckel, Norbert
Lissat, Andrej
von Stackelberg, Arendt
Thieme, Nadine
Doueiri, Mohemed-Salim
Spors, Birgit
Beck-Broichsitter, Benedicta
Heiland, Max
Raguse, Jan-Dirk
author_facet Neckel, Norbert
Lissat, Andrej
von Stackelberg, Arendt
Thieme, Nadine
Doueiri, Mohemed-Salim
Spors, Birgit
Beck-Broichsitter, Benedicta
Heiland, Max
Raguse, Jan-Dirk
author_sort Neckel, Norbert
collection PubMed
description Langerhans cell histiocytosis (LCH) is a diagnostic and therapeutic challenge. We report on a rare case of its primary oral manifestation that was treated successfully with the BRAF-specific agent, vemurafenib, after insufficient standard LCH treatment. This case underlines the importance of proper diagnosis and the evaluation of targeted therapy as a valuable tool in LCH treatment. Furthermore, the close collaboration of surgeons, oncologists, and dentists is mandatory to ensure adequate treatment, restore the stomatognathic system in debilitating post-treatment situations, improve quality of life, and ensure effective disease control in infants and young patients.
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spelling pubmed-68018822019-10-30 Primary oral manifestation of Langerhans cell histiocytosis refractory to conventional therapy but susceptible to BRAF-specific treatment: a case report and review of the literature Neckel, Norbert Lissat, Andrej von Stackelberg, Arendt Thieme, Nadine Doueiri, Mohemed-Salim Spors, Birgit Beck-Broichsitter, Benedicta Heiland, Max Raguse, Jan-Dirk Ther Adv Med Oncol Case Report Langerhans cell histiocytosis (LCH) is a diagnostic and therapeutic challenge. We report on a rare case of its primary oral manifestation that was treated successfully with the BRAF-specific agent, vemurafenib, after insufficient standard LCH treatment. This case underlines the importance of proper diagnosis and the evaluation of targeted therapy as a valuable tool in LCH treatment. Furthermore, the close collaboration of surgeons, oncologists, and dentists is mandatory to ensure adequate treatment, restore the stomatognathic system in debilitating post-treatment situations, improve quality of life, and ensure effective disease control in infants and young patients. SAGE Publications 2019-10-19 /pmc/articles/PMC6801882/ /pubmed/31666812 http://dx.doi.org/10.1177/1758835919878013 Text en © The Author(s), 2019 http://www.creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Neckel, Norbert
Lissat, Andrej
von Stackelberg, Arendt
Thieme, Nadine
Doueiri, Mohemed-Salim
Spors, Birgit
Beck-Broichsitter, Benedicta
Heiland, Max
Raguse, Jan-Dirk
Primary oral manifestation of Langerhans cell histiocytosis refractory to conventional therapy but susceptible to BRAF-specific treatment: a case report and review of the literature
title Primary oral manifestation of Langerhans cell histiocytosis refractory to conventional therapy but susceptible to BRAF-specific treatment: a case report and review of the literature
title_full Primary oral manifestation of Langerhans cell histiocytosis refractory to conventional therapy but susceptible to BRAF-specific treatment: a case report and review of the literature
title_fullStr Primary oral manifestation of Langerhans cell histiocytosis refractory to conventional therapy but susceptible to BRAF-specific treatment: a case report and review of the literature
title_full_unstemmed Primary oral manifestation of Langerhans cell histiocytosis refractory to conventional therapy but susceptible to BRAF-specific treatment: a case report and review of the literature
title_short Primary oral manifestation of Langerhans cell histiocytosis refractory to conventional therapy but susceptible to BRAF-specific treatment: a case report and review of the literature
title_sort primary oral manifestation of langerhans cell histiocytosis refractory to conventional therapy but susceptible to braf-specific treatment: a case report and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6801882/
https://www.ncbi.nlm.nih.gov/pubmed/31666812
http://dx.doi.org/10.1177/1758835919878013
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