Giant cell tumor of bone: updated molecular pathogenesis and tumor biology

Giant cell tumor of bone (GCTB)–related clonal aberrations occur in a background of epigenetic histone modifications (especially the G34W mutation of H3F3A gene) that induce cytogenetic abnormalities. Clonal aberrations are closely linked to the aggressiveness of GCTB. The “neoplastic” mononuclear s...

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Bibliographic Details
Published in:Human pathology 2018-11, Vol.81, p.1-8
Main Authors: Noh, Byeong-Joo, Park, Yong-Koo
Format: Article
Language:English
Subjects:
Biology
Bone cancer
Bones
Chemokines
Chromosomes
Cysts
Cytokines
Epigenetics
Fibroblasts
G34W mutation
Genes
Giant cell tumor of bone
H3F3A
Molecular pathogenesis
Morphology
Mutation
Pathogenesis
Proteins
RANKLs
Tumor microenvironment
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Summary:Giant cell tumor of bone (GCTB)–related clonal aberrations occur in a background of epigenetic histone modifications (especially the G34W mutation of H3F3A gene) that induce cytogenetic abnormalities. Clonal aberrations are closely linked to the aggressiveness of GCTB. The “neoplastic” mononuclear stromal cells in GCTB express fundamental RANKLs and various chemokines and cytokines associated with monocyte recruitment and “reactive” multinucleated giant cells (osteoclastogenesis). The reciprocal and orchestrated actions between mononuclear stromal cells and multinucleated giant cells help in the understanding of the molecular pathogenesis and tumor biology of GCTB. In the future, novel targets in the updated tumor biology and molecular pathogenesis of GCTB should be explored and scrutinized for the development of systemic therapy. •Ginat cell tumor of bone (GCTB)-related clonal aberrations occur in a background of epigenetic histone modifications that induce cytogenetic abnormalities.•Clonal aberrations are closely linked to the aggressiveness of GCTB.•The “neoplastic” mononuclear stromal cells express fundamental RANKLs and various chemokines and cytokines.•RANKL expression and secreted variable chemo- and cytokines induce monoytic recruitment and “reactive” multinucleated giant cells.•The orchestrated actions between “neoplastic” mononuclear stromal cells and “reactive” multinucleated giant cells helps in the understanding of the molecular pathogenesis and tumor biology of GCTB.
ISSN:0046-8177
1532-8392
DOI:10.1016/j.humpath.2018.06.017