Cherubism – new hypotheses on pathogenesis and therapeutic consequences

Clinico-Pathologic Conference AAOMP/IAOP 2008: Case 3

“…This long-time known observation has been recently explained on the basis of a spatio-temporal association between cherubism and the failure of development of the second and third permanent mandibular molars [3]. The histopathological findings in cherubism are essentially similar but not entirely identical to those seen in CGCG, the main difference lies in the presence of perivascular eosinophilic cuff-like deposits in the former.…”

So-called Hybrid Central Odontogenic Fibroma/Central Giant Cell Lesion of the Jaws. A Report on Seven Additional Cases, Including an Example in a Patient with Cherubism, and Hypotheses on the Pathogenesis

“…Taking this into consideration it can be speculated that the CGCL of cherubism induced a COF-like proliferation. In cherubism, it has been proposed that mutation in the gene encoding the SH3-binding protein (SH3BP2) may induce delayed imbalance of factors participating in odontogenesis [20], in particular upregulation of Msx-1 that can stimulate proliferation of residual odontogenic epithelium. Increased Msx-1 expression of the same order as for tooth germs has been found in CGCL [20].…”

Increased expression of NFATc1 in giant cell lesions of the jaws, cherubism and brown tumor of hyperparathyroidism

“…The disease is characterized by bilateral expansion of the maxilla and/or mandible, usually detected in early childhood, and shows progressive growth until puberty (4). BTH is a nonneoplastic lesion resulting from abnormal bone metabolism in hyperparathyroidism and may be associated with the primary or secondary types of the disease (5).…”