Year : 2018

Volume : Volume 9

Issue : Issue 1

Address for correspondence :
Dr. Anish Tiwari.
dr.anish.ti@gmail.com

Abstract

Background: In Indian scenario, Gorlin–Goltz syndrome (nevoid basal cell carcinoma syndrome [NBCCS]) has been rarely reported. The clinical, radiological, and histopathological findings and major and minor criteria in five cases of NBCCS in North Indian population have been presented along with a discussion of the role of gene mutation analysis in early diagnosis of syndrome. It is an infrequent multisystemic disease with an autosomal dominant trait, with complete penetrance and variable expressivity, though sporadic cases have been described. This article includes a case report and an extensive review of the GGS with regard to its history, incidence, etiology, features, investigations, diagnostic criteria, keratocystic odontogenic tumor and treatment modalities.

Keywords: Goltz-Gorlin syndrome, keratocystic odontogenic tumor, odontogenic keratocyst.