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KMID : 0360120120340050357
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Journal of the Korean Society of Maxillofacial Plastic Reconstructive Surgeons
2012 Volume.34 No. 5 p.357 ~ p.362
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Cherubism: Review of 2 Cases
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Kwak Ju-Hee
Kim Ji-Youn
Kim Myung-Rae
Kim Sun-Jong
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Abstract
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Cherubism is a rare familial disease of childhood, characterized by proliferative lesion, which is within the maxilla and mandible. In a typical case, painless symmetric expansile lesions develop in the jaws. It shows substitution of the bone by proliferating fibrous tissue exhibiting mature fibroblasts and a number of multinucleated giant cells within an intercellular matrix. Usually, the disease manifests in early childhood, and becomes more marked until puberty, at which time the bony lesions begin to regress. As such, conservative approaches to management are advisable. However, excision of tissue through enucleation or curettage appears to be necessary in more aggressive cases, to reduce the maxillofacial deformity after puberty and to ensure a successful outcome without the risk of progression, requiring additional resection. This report describes 2 cases of manifestation of cherubism of oral and maxillofacial region. We present diagnosis, radiological - histopathologic features, and treatment of cherubism.
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KEYWORD
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Cherubism, Giant cells, Genes dominant, Maxilla, Mandible
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