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

Norbert Neckel,Andrej Lissat, Arendt Von Stackelberg,Nadine Thieme, Mohemed-Salim Doueiri,Birgit Spors, Benedicta Beck-Broichsitter,Max Heiland,Jan-Dirk Raguse

THERAPEUTIC ADVANCES IN MEDICAL ONCOLOGY(2019)

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摘要
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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关键词
BRAF inhibitor,BRAF mutation,BRAF V600E-targeted therapy,dental and oral rehabilitation,Langerhans cell histiocytosis (LCH),oral lesions,oral ulcers,vemurafenib
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