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Imaging characteristics of cherubism
Francesca D Beaman1, Laura W Bancroft, Jeffrey J Peterson
1Department of Radiology, Mayo Clinic, 4500 San Pablo Rd., Jacksonville, FL 32224-3899, USA.
AJR. American Journal of Roentgenology
|March 25, 2004
Summary
Cherubism, a rare pediatric bone disorder, presents with distinctive jaw imaging. Key features include symmetric jaw expansion, thin cortexes, and multilocular radiolucencies, aiding diagnosis.
Area of Science:
- Pediatric Radiology
- Skeletal Dysplasias
- Genetic Bone Disorders
Background:
- Cherubism is a rare, benign, autosomal dominant genetic disorder.
- It primarily affects the craniofacial bones, particularly the mandible and maxilla.
- Clinical manifestations often appear in early childhood.
Purpose of the Study:
- To delineate the characteristic radiographic and advanced imaging findings of cherubism.
- To assist clinicians in differentiating cherubism from other jaw pathologies.
- To highlight imaging features that support the diagnosis.
Main Methods:
- Review of radiographic and advanced imaging studies (CT, MRI) in patients with confirmed cherubism.
- Analysis of lesion morphology, distribution, and associated bone changes.
- Correlation of imaging findings with clinical and histopathological data.
Main Results:
- Bilateral, relatively symmetric expansile lesions of the maxilla and mandible are typical.
- Imaging reveals thinning of the cortical bone with internal multilocular radiolucencies.
- A coarse, trabecular pattern within the lesions is commonly observed.
- Lesions are generally well-defined and may displace tooth buds.
Conclusions:
- Cherubism exhibits characteristic, though not pathognomonic, radiographic features.
- Symmetric jaw involvement limited to the maxilla and mandible strongly suggests the diagnosis.
- Imaging findings of expansile remodeling, cortical thinning, and multilocular radiolucencies are key diagnostic indicators.