Cherubism: A Clinical, Radiographic, and Histopathologic Comparison of 7 Cases
Purpose
Cherubism is an uncommon fibro-osseous disorder of the jaws that presents with varying degrees of involvement and a tendency toward spontaneous remission. Lesions are characterized by replacement of bone with fibrovascular tissue containing abundant multinucleated giant cells. We attempted to study the relationships among the degree of cherubism, the radiographic extent of the jaw lesions, the histopathologic findings, and the clinical course of 7 patients.
Patients and Methods
In 7 patients diagnosed with cherubism, we evaluated the degree of fibrosis and perivascular cuffing, the presence of focal hemosiderin deposits, and giant multinucleated cell density (absent, few, moderate, or severe). Clinical course and progression were also assessed using a 4-point scale (improvement, no changes, modest progression, and marked progression).
Results
The patients were followed up for an average of 8.5 years. Two patients exhibited clinical and radiographic improvement, while 3 showed no changes, and 2 progressed despite surgical treatment in 1 of them.
Conclusion
The course of cherubism in 1 of our patients may represent evidence of an association between the presence of abundant multinucleated giant cells, an increased extent of the lesions, and a more aggressive behavior of the disease.
⁎Associate Professor of Oral Surgery, Faculty of Medicine and Dentistry, Valencia University, Valencia, Spain
†Section Chief of Maxillofacial Surgery, “La Fe” University Hospital Children’s Center, Valencia, Spain
‡Consultant in Maxillofacial Surgery, “La Fe” University Hospital Children’s Center, Valencia, Spain
§Full Professor of Oral Medicine, Faculty of Medicine and Dentistry, Valencia University; Head of the Service of Stomatology, Valencia University General Hospital, Valencia, Spain
¶Associate Professor of Pathology, Faculty of Medicine and Dentistry, Valencia University; Chairman of Pathology II, “La Fe” University Hospital, Valencia, Spain
∥Professor of the Master in Oral Surgery, Faculty of Medicine and Dentistry, Valencia University, Valencia, Spain
Address correspondence and reprint requests to Dr Peñarrocha: Unidad Médico-Quirúrgica, Gascó Oliag 1, 46021 Valencia, Spain
ABSTRACT