• Matthias Papo, Fleur Cohen-Aubart, Ludovic Trefond, Adeline Bauvois, Zahir Amoura, Jean-François Emile, and Julien Haroche.
• Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 2, Centre National de Références des Histiocytoses, Hôpital Pitié-Salpêtrière, Sorbonne Université, 47-83, Boulevard de l'Hôpital, 75651, Paris Cedex 13, France.
• Curr Oncol Rep. 2019 May 21; 21 (7): 62.

Purpose Of ReviewProvide an overview of recent progress in decoding the pathogenesis and treatment of systemic histiocytoses.Recent FindingsAdvances in molecular techniques over the last few years, enabling the identification of several MAPK mutations in lesion histiocytes, have revolutionized our understanding of histiocytosis that led to a revised classification and new treatments. Since the 2010 discovery of the BRAFV600E mutation in 57% of Langerhans cell histiocytosis (LCH) lesions, several other kinase mutations have been found, mostly in the MAPK pathway, and also in other key signaling pathways, in LCH, Erdheim-Chester Disease (ECD) and, less frequently, Destombes-Rosai-Dorfman disease (RDD). Those revolutionary breakthroughs enhanced our understanding of the pathogenesis of histiocytosis and led to trials with targeted therapies that demonstrated notable efficacy.

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