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

Autor: Shyam Kishor Sah, Shyama Jain, Ernst J Reichenberger, Khushboo Singh, Anju Garg, Seema Kapoor, Sunita Gupta, Nirupama Trehanpati, P.S. Bhandari
Rok vydání: 2019
Předmět:
Zdroj: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology. 128:e191-e201
ISSN: 2212-4403
Popis: Cherubism 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.
Databáze: OpenAIRE