Systemic Histiocytosis (Langerhans Cell Histiocytosis, Erdheim –Chester Disease, Destombes–Rosai–Dorfman Disease): from Oncogenic Mutations to Inflammatory Disorders

AbstractPurpose 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.SummarySince the 2010 discovery of theBRAFV600E 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.
Source: Current Oncology Reports - Category: Cancer & Oncology Source Type: research