Clinicopathologic study of 24 cases of cherubism.
The authors reviewed 24 cases of familial or nonfamilial cherubism. The age at onset was between 6 and 10 years. It was characterized by bilateral painless swelling of jaws and eyes-to-heaven appearance was visible when the maxillae were affected as well. Radiographs showed well-defined multilocular radiolucencies and with age, thick sclerotic borders were visible. 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 apparent. Multinucleated giant cells were positive for osteoclastic specific markers, tartrate-resistant acid phosphatase and human alphaV beta3 integrin, 23C6. Results after follow-up were available for 14 cases. Of these, no treatment was carried out in five cases, cherubism resolved (three cases) or grew slowly (two cases); curettage or surgical contouring was performed in seven cases, during the rapid growth of the lesions. This not only gave good immediate results, but also arrested active growth of remnant cherubic lesions and even stimulated bone regeneration. Segmental mandibulectomy followed by reconstruction was performed in two cases with extensive lesion and the risk of pathologic fracture of the mandible, and excellent results were obtained.
Meng
X-M
XM
Department of Oral Pathology, Peking University School of Stomatology, Haidian District, Beijing 100081, PR China. m.xm@163.com
Yu
S-F
SF
Yu
G-Y
GY
D048868:Adaptor Proteins, Signal Transducing / Q000235:genetics; D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D019943:Amino Acid Substitution; D002636:Cherubism / Q000097:blood / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D005799:Genes, Dominant; D015726:Giant Cells / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007568:Jaw / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D056948:Orthognathic Surgical Procedures; D010375:Pedigree; D011862:Radiography, Panoramic
D048868:Adaptor Proteins, Signal Transducing; C109073:SH3BP2 protein, human