Clinicoradiologic follow up of cherubism with aggressive characteristics: a series of 3 cases

Publication date: Available online 7 February 2019Source: Oral Surgery, Oral Medicine, Oral Pathology and Oral RadiologyAuthor(s): Sunita Gupta, Khushboo Singh, Anju Garg, P.S. Bhandari, Shyam Kishor Sah, Ernst Reichenberger, Seema Kapoor, Shyama Jain, Nirupama TrehanpatiCherubism is a rare autosomal dominant disorder characterized by replacement of bone with fibrous tissue containing multinucleated giant cells. It manifests as bilateral mandibular and/or maxillary enlargement. The 2017 World Health Organization classification lists cherubism as a giant cell lesion of the jaws, distinct from fibro-osseous disorders. We discuss 3 cases of familial cherubism having aggressive characteristics and present clinicoradiologic evaluations of the lesions over 12, 18, and 1.5 years, respectively. Follow-up was observational, without active intervention. Analysis of the lesions for change in size and functional impairments was correlated with periodic imaging. All patients are currently being monitored. The outcome in 2 cases has been excellent without intervention, but 1 case had extensive involvement of the jaws and involvement of the condyle and orbit. A secondary giant cell lesion involved the palate in one patient's mother, who had had cherubic lesions in childhood.
Source: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology - Category: ENT & OMF Source Type: research