Nevoid basal cell carcinoma syndrome in Indian patients: a clinical and radiological study of 6 cases and review of literature

Abstract

Objective: Nevoid basal cell carcinoma syndrome (NBCCS) is rarely reported in the Indian population. We present the clinical and radiological features of 6 cases of NBCCS in Indian patients.

Study design: The clinical and radiological features of 6 cases of NBCCS were characterized into major and minor criteria and compared with features reported in Indian patients and in patients from other parts of the world.

Results: The most common features seen were presence of multiple keratocystic odontogenic tumors, rib abnormalities, and calcification of falx cerebri. Talons cusp and supernumerary teeth are features not previously reported in association with NBCCS. Basal cell carcinoma was not seen in any patient.

Conclusions: Combining the current series with 17 additional cases reported in Indian patients, it is evident that the frequency of clinical and radiological features in NBCCS in Indian patients differs from other ethnic groups.