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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
Author(s) -
Neckel Norbert,
Lissat Andrej,
von Stackelberg Arendt,
Thieme Nadine,
Doueiri Mohemed-Salim,
Spors Birgit,
Beck-Broichsitter Benedicta,
Heiland Max,
Raguse Jan-Dirk
Publication year - 2019
Publication title -
therapeutic advances in medical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.272
H-Index - 49
eISSN - 1758-8359
pISSN - 1758-8340
DOI - 10.1177/1758835919878013
Subject(s) - medicine , langerhans cell histiocytosis , vemurafenib , dermatology , refractory (planetary science) , disease , histiocytosis , quality of life (healthcare) , rare disease , intensive care medicine , pathology , cancer , physics , astrobiology , nursing , metastatic melanoma
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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