Open AccessHistiocytic neoplasms: modern aspects of pathogenesis, classification, diagnosis and treatment
Author(s) -
О. D. Gurieva,
Т. Т. Валиев,
A. I. Pavlovskaya
Publication year - 2022
Publication title -
onkogematologiâ
Language(s) - English
Resource type - Journals
eISSN - 2413-4023
pISSN - 1818-8346
DOI - 10.17650/1818-8346-2022-17-1-10-25
Subject(s) - histiocyte , juvenile xanthogranuloma , histiocytosis , langerhans cell histiocytosis , erdheim–chester disease , pathogenesis , pathological , pathology , medicine , somatic cell , differential diagnosis , disease , cancer research , biology , gene , genetics
Histiocytic cell tumors present as a difficult group of diseases in terms of differential diagnosis due to their rare frequency, heterogeneous clinical manifestations and ambiguous clinical course. Considering MAPK-signaling pathway mutations role (including BRAFV600E) in the pathogenesis of histiocytic cell tumors the importance of genetic studies in the diagnosis and choice of therapeutic strategy in these diseases increases. In the current issue modern classifications of histiocytic cell tumors, morphological and immunophenotypic features of histiocytic and dendritic cells, and the role of recurrent somatic mutations of the MAPK signaling pathway in the pathological histiocytic proliferation are presented. Particular attention is given to Langerhans cell histiocytosis, Erdheim–Chester disease and juvenile xanthogranuloma. Clinical and diagnostic characteristics of these diseases and therapeutic approaches are presented.