Nevoid basal cell carcinoma syndrome: A case report

Abstract

Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant inherited condition that exhibits high penetrance and variable expressivity. The syndrome is caused by mutations in patch (PTCH), a tumor suppressor gene that has been mapped to chromosome 9q22.3-q31. NBCCS is characterized by basal cell carcinomas (BCCs), odontogenic keratocysts of the jaws, pits of the palms and soles, ectopic calcification particularly of the falx cerebri, and skeletal anomalies. Other features, including ovarian fibromas, medulloblastoma, ocular anomalies, and neurological defects, are also associated with this syndrome. It arises in all races with equal sexual predilection. It is a rare syndrome and incidence rate is 5%. In this report, we present a case of NBCCS in a 30-year-old male patient.

Keywords

• Basal cell nevus syndrome
• Gorlin syndrome
• Gorlin–Goltz syndrome
• nevoid basal cell carcinoma syndrome
• odontogenic keratocyst