Giant cell reparative granuloma of the orbit associated with cherubism

PURPOSE: To report a case of cherubism with extensive, bilateral orbital involvement occurring in a 27-year-old woman who had the diagnosis established at the age of 4 years. DESIGN: Single interventional case report. INTERVENTION: Ophthalmologic examination and computed tomography were performed. The patient underwent multiple surgical excisions using a bicoronal and transorbital approach. The excised orbital tissues were studied histopathologically. RESULTS: Computed tomography showed bilateral inferior lateral masses involving the orbital floors and producing marked superior displacement of the orbital contents. The intrinsic expansile bone lesions involved the inferior and lateral orbital walls with apical compression of the optic nerves. Histopathologic examination of the masses revealed scattered giant cells in a fibroblastic stroma containing small vascular channels. The lesion was interpreted as giant cell reparative granuloma. CONCLUSIONS: Giant cell reparative granuloma is an uncommon bone lesion that might involve the orbit. Cherubism should be included in the differential diagnosis of lesions that show the histopathologic features of giant cell reparative granuloma.