[Clinicopathologic study of a family cherubism].
OBJECTIVE
To investigate the clinicopathologic features of familial cherubism and its differentiation from other giant cell lesions in jaws and the results of surgical treatments with a long-term follow-up.


METHODS
Four cases of familial cherubism were reviewed and their clinical and radiographic features, histopathologic appearance, biochemical markers and surgical treatments analysed.


RESULTS
Clinically, cherubism was characterized by bilateral painless swelling of jaws, mandibular deformity was common. Radiographs showed multilocular radiolucencies with sclerotic thickening border. 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. The lesion regressed without treatment in 1 cases. Curettage was performed in 3 cases with good results.


CONCLUSIONS
Cherubism can be diagnosed according to its typical clinical and radiographical features with a positive family history. It might regress without treatment. But surgery intervention is suggested to improve physiological function and to solve the psychologic problem of the patients.
Meng
Xue-Mei
XM
Department of Pathology, Peking University School of Stomatology, Beijing 100081, China. m.xm@163.com
Yu
Shi-Feng
SF
Wu
Yun-Tang
YT
D000328:Adult; D002636:Cherubism / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D006801:Humans; D008297:Male; D010375:Pedigree