Prikaz rijetkog slučaja sporadične ponovne pojave kerubizma

Abstract

Cherubism is a rare bone dysplasia in children characterized by symmetrical bone resorption limited only to the jaws and diagnosed as bone lesions filled with soft fibrous giant cell-rich tissue that can expand and cause severe facial deformity. Most patients have germline mutations in the gene encoding SH3BP2, a signaling adaptor protein involved in adaptive and innate immune responses. Treatment depends on the clinical course of the disease. The current case report presents a young patient with cysts in frontal region of maxilla without family history, that had recurrences of cysts, and is representing a less documented, rare case. Histological finding at that time was indicating a giant cell granuloma and later a grey tumor. Due to the existence of other pathological findings in the jaws with the presence of giant cells, we had difficulties to diagnose cherubism as surgeons.