Open AccessGiant Cell Lesions of the Jaws Involving RASopathy Syndromes
Author(s) -
Melissa J. Luna,
Nicholas Wolsefer,
Carlos-Xavier Zambrano,
Ivan J. Stojanov
Publication year - 2022
Publication title -
acta stomatologica croatica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.391
H-Index - 9
eISSN - 1846-0410
pISSN - 0001-7019
DOI - 10.15644/asc56/1/9
Subject(s) - cherubism , medicine , noonan syndrome , neurofibromatosis , craniofacial , central giant cell granuloma , dermatology , pathology , giant cell , psychiatry
Giant cell lesions of the jaws (GCLJ) may rarely occur in the setting of RASopathy syndromes such as Noonan syndrome or neurofibromatosis I. Recently, central giant cell granulomas (CGCG), the most common of the GCLJ, have been recognized as benign neoplasms characterized by Ras/MAPK signaling pathway mutations. This provides a rational basis for understanding GCLJ in RASopathy syndromes as syndromically occurring CGCG. This review aims to summarize the clinicopathologic features of syndromic CGCG and to review the salient clinical and craniofacial features of the syndromes in which they may rarely occur.