A family of familial gigantiform cementoma: clinical study

Abstract

Background

Familial gigantiform cementoma is an autosomal dominant fibro-cement osseous lesion that causes massive expansion of facial skeleton. Patients with such massive lesions have a compromised quality of life. The main goal of treating such patients is to restore and preserve the jaw as far as possible which would enhance their quality of life.

Purpose

This study was conducted to identify the occurrence of gigantiform cementoma which had affected three generations of a family and also to focus on documentation of the clinical course and management.

Method

Patients (one family—mother, grandmother, aunt and grandson) who had visited the Department of Oral and Maxillofacial Surgery, Tamilnadu Government Dental College and Hospital, Chennai, over a period for their swelling in the maxillofacial region were clinically and radiographically evaluated and histopathologically diagnosed as familial gigantiform cementoma; later, they were surgically managed.

Result

It is very rare to document three generations of this disease which had shown varied clinical presentation (asymptomatic slow growth, arrested growth and one case of aggressive growth). Management of these cases varied from observation to aggressive resection.

Conclusion

Gigantiform cementoma follows an autosomal dominant pattern of inheritance with variable phenotypic expression without gender predilection. These cases require regular observation and intervention if necessary.