CLINICO-RADIOLOGICAL FOLLOW UP OF CHERUBISM WITH AGGRESSIVE CHARACTERISTICS: A SERIES OF 3 CASES

Publication date: Available online 7 February 2019

Source: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology

Author(s): Dr. Sunita Gupta, Dr. Khushboo Singh, Dr. Anju Garg, Dr. P.S. Bhandari, Dr. Shyam Kishor Sah, Dr. Ernst Reichenberger, Dr. Seema Kapoor, Dr. Shyama Jain, Dr. Nirupama Trehanpati

ABSTRACT

Background

Cherubism is a rare autosomal dominant disorder characterized by degradation of bone that is replaced by fibrous tissue containing multinucleated giant cells. It clinically manifests as bilateral mandibular and maxillary enlargement. The recent World Health Organization (WHO) classification of 2017 now lists cherubism as one of the giant cell lesions of the jaws, distinct from fibro-osseous disorders.

Materials and Methods

We discuss three cases of familial cherubism having aggressive characteristics with clinico-radiologic evaluation of the lesions for 12 years, 18 years, and 1.5 years, respectively. Follow-up was observational without active intervention. Clinical analysis of the lesions for increase or decrease in size and association with functional impairments was correlated with periodic conventional and advanced imaging. All three cases are currently on a follow-up examination schedule.

Conclusion

The outcome in two cases with long term follow-up has been excellent without intervention, although one case had extensive involvement of the jaws as well as unusual involvement of the mandibular condyle and orbit. Another unique finding was the development of a secondary giant cell lesion involving the palate in the affected mother of patient 3, who had had cherubic lesions in childhood.

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