A Paradigm Shift in the Management of Cherubism? A preliminary report using Imatinib

Cherubism is a skeletal dysplasia caused by a mutation of SH3BP2 gene. This leads to bilateral symmetric fibro osseous lesions of the maxilla and mandible. Initial jaw expansion typically begins during the toddler years, with progression through puberty. After puberty the lesions usually involute and disappear by adulthood. Therefore, surgery is typically reserved for the most symptomatic or disfiguring cases. Reported symptoms include pain, difficulty with mastication, and airway compromise. Once symptoms present, definitive management options are few, and may carry great risks.

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