A Rare Case Report of Non-Familial Sporadic Reoccurrence of Cherubism: Difficulties from the Surgeon’s Point of View

Vjosa Hamiti Krasniqi, Mergime Prekazi Loxha, Zana Agani, Aida Rexhepi, Jehona Ahmedi, David Stubljar

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 the 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 existence of other pathological findings in jaws with the presence of giant cells, we had difficulties to diagnose cherubism as surgeons.

doi:10.5671/ca.46.2.9


Keywords*


cherubism; jaw tumors; mandibula; maxilla; case report

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