Systemic Histiocytosis (Langerhans Cell Histiocytosis, Erdheim-Chester Disease, Destombes-Rosai-Dorfman Disease): from Oncogenic Mutations to Inflammatory Disorders.
Curr Oncol Rep
; 21(7): 62, 2019 05 21.
Article
in En
| MEDLINE
| ID: mdl-31115724
ABSTRACT
PURPOSE OF REVIEW Provide an overview of recent progress in decoding the pathogenesis and treatment of systemic histiocytoses. RECENT FINDINGS:
Advances 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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Histiocytosis, Sinus
/
Histiocytosis, Langerhans-Cell
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Erdheim-Chester Disease
/
Proto-Oncogene Proteins B-raf
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Protein Kinase Inhibitors
/
Inflammation
/
Mutation
Type of study:
Prognostic_studies
Limits:
Humans
Language:
En
Journal:
Curr Oncol Rep
Journal subject:
NEOPLASIAS
Year:
2019
Type:
Article
Affiliation country:
France