Multiple versus Solitary Giant Cell Lesions of the Jaw: Similar or Distinct Entities?

摘要: Abstract The majority of giant cell lesions the jaw present as a solitary focus disease in bones maxillofacial skeleton. Less frequently they occur multifocal lesions. This raises clinical dilemma if these should be considered distinct entities and therefore each need specific therapeutic approach. Solitary with great diversity symptoms. Recent molecular analysis revealed that are associated somatic gain-of-function mutations KRAS, FGFR1 or TRPV4 large component mononuclear stromal cells which all act on RAS/MAPK pathway. For lesions, small group neoplastic remain after ruling out hyperparathyroidism. Strikingly, most patients diagnosed before age 20 years, thus completion dental development. These often accompanied by diagnosis strong suspicion syndrome. Many reported syndromes belong to so-called RASopathies, germline mosaic leading downstream upregulation other syndrome is cherubism, SH3BP2 gene through assumed unknown signaling an autoinflammatory bone disorder hyperactive osteoclasts defective osteoblastogenesis. Based this extensive literature review, pathway activation hypothesized jaw. different interaction between contribution deregulated individual lineages crosstalk pathways among germline- non-germline-based alterations causing can explanatory for characteristic features. As such, might also aid understanding age-dependent symptomatology jaw; hopefully guiding ideal timing when installing treatment strategies future.