Anti-tumor necrosis factor treatment in cherubism - Clinical, radiological and histological findings in two children

► Cherubism is a rare a disfiguring genetic disorder with excessive bone resorption and multilocular lesions in the mandible and/or maxilla. ► Findings in the cherubism mouse model suggest that removal of TNF-α prevents the development of the bone phenotype. ► We treated two children with active cherubism with the TNF-α antagonist adalimumab for approximately 2.5 years. ► Adalimumab appeared to decrease the formation of pathogenic giant cells, but did not induce lesion regression or prevent lesion expansion. ► TNF-α antagonist treatment does not appear to provide sufficient amelioration for patients suffering from cherubism.