Hosting 718 files, 2916 images, and 4 contributors.

 
Random Case

Congenital Pseudocyst
Joseph Junewick, MD FACR
over 6 years ago
Enter A Workflow
Standard2   Academic2

Please choose a workflow. A standard workflow allows you to browse the repository with full case detail; the academic workflow allows you to browse the repository with limited case detail revealed. Double click on the images to launch image viewer.

ARSt Case Repository

Fibrous Dysplasia - Cherubism

Case Detail

Anatomy: Neck-Face
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 4 years ago
Updated: over 4 years ago
Tags: PEDS
Modality/Study Types: CT
Activities:
Adobe32 PDF Imagej32 ImageJA

History

2 year old with sinusitis and broad nasal bridge.


Case Images


Diagnosis

Fibrous Dysplasia – Cherubism

Findings

CT – Axial and coronal noncontrast images show symmetric expansion of the maxillae and mentum of the mandible with “cotton-wool” matrix.

Discussion

Cherubism is a subtype of fibrous dysplasia and the result of a point mutation of the SH-3 binding protein on the short arm of chromosome 4 (4p16.3). Radiographically cherubism is characterized as expansile remodeling of the maxilla and/or mandible with mild sclerosis, distorganized trabeculation and absence of periostitis. Differential diagnosis includes craniofacial fibrous dysplasia, brown tumor of hyperparathyroidism, Jaffe-Campanacci syndrome, and familial gigantiform cementoma. Although cherubism and craniofacial fibrous dysplasia of individual lesions show radiologic similarities, they may be distinguished clinically and histologically. Features more specific for the diagnosis of cherubism include bilateral mandibular involvement, limitation to the maxilla and mandible, and involution at the time of puberty. In contrast, patients with fibrous dysplasia typically do not present with swollen cheeks, upward turning of the eyes, or dental derangement. Histologically, patients with cherubism typically have a prominent number of multinucleated giant cells, which are rarely seen in fibrous dysplasia. Brown tumor and Jaffe-Campanacci syndrome are readily distinguished on clinical grounds and are easily eliminated from the differential diagnosis. Familial gigantiform cementoma is usually located primarily in the maxilla and focal rather than diffuse.

Reference

Beaman FD, Bancroft LW, Peterson JJ et al. Imaging characteristics of cherubism. AJR (2004); 182(4): 1051-1054.



Corporate Office: 616.363.7272, 3264 North Evergreen Drive, Grand Rapids, MI 49525

Spectrum Health Helen Devos Childrens Hospital GE HealthCare