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

doi:10.1177/1758835919878013

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

Ther Adv Med Oncol. 2019 Oct 19:11:1758835919878013. doi: 10.1177/1758835919878013. eCollection 2019.

Authors

Norbert Neckel¹, Andrej Lissat², Arendt von Stackelberg², Nadine Thieme³, Mohemed-Salim Doueiri⁴, Birgit Spors⁵, Benedicta Beck-Broichsitter⁶, Max Heiland⁶, Jan-Dirk Raguse⁶

Affiliations

¹ Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Oral and Maxillofacial Surgery, Augustenburger Platz 1, 13351 Berlin 13353, Germany.
² Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Paediatrics, Division of Oncology and Haematology, Berlin, Germany.
³ Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Radiology, Berlin, Germany.
⁴ Charité - Universitätsmedizin Berlin, Centre for Dental and Craniofacial Sciences, Department of Orthodontics, Dentofacial Orthopaedics and Paedodontics, Berlin, Germany.
⁵ Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Paediatric Radiology, Berlin, Germany.
⁶ Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Oral and Maxillofacial Surgery, Berlin, Germany.

Abstract

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.

Keywords: BRAF V600E-targeted therapy; BRAF inhibitor; BRAF mutation; Langerhans cell histiocytosis (LCH); dental and oral rehabilitation; oral lesions; oral ulcers; vemurafenib.

Publication types

Case Reports