Clinicopathologic study on 24 cases of cherubism

Objective: To investigate the clinical, radiographic features, histopathologic appearance, biochemical markers of cherubism and its diagnosis criteria to differentiate from other giant cell lesions in jaws. Methods: 24 cases of familial or nonfamilial cherubism were reviewed in the clinical, radiographic features, histopathologic appearance, biochemical markers and were evaluated in the relation among its severity, the nature course and the types of surgery intervention. Paraffin-embedded specimens were used to detect the expression of tartrate-resistant acid phosphatase (TRAP) and frozen specimens were used to detect the expression of the vitronectin receptor (VNR), av b3 integrin by using immunohistochemical method (SP) to determine the characterization of multinucleated giant cells. Results: The age at onset of the patients was between 6 and 10 years old. 14 are familial. Clinically, it was characterized of bilateral painless swelling of jaws (22/24), mandibular deformity was common and in 3 severe cases,eyes-to-heaven appearance was visible when maxillae were affected. Radiographs showed multilocular radiolucencies with sclerotic thickening border with age. A malocclusive and abnormal dentition, worse in the mandible can be seen. Histopathologically, numerous randomly distributed multinucleated giant cells and vascular spaces within a fibrous connective tissue stroma with or without eosinophilic collagen perivascular cuffing were shown. Multinucleated giant cells were positive for osteoclastic specific markers, TRAP and VNR. Cherubism regressed without treatment in 3 cases. Curettage was performed in 5 cases, osteoplasty was performed in 2 cases with the rapid growth of the lesions, they not only gave a good immediate result, but also arrested active growth of remnant cherubic lesions and even stimulated bone regeneration. Conclusions: Radiographically, Cherubism shows multilocular radiolucencies with sclerotic thickening borders; Histologically, the lesion contains numerous multinucleated giant cells scattered throughout a fibrous connective tissue stroma .It might regress without treatment.