Nevoid Basal Carcinoma Syndrome Gorlin-Goltz Syndrome): A Case Report and Literature Review

Abstract

Nevoid basal cell carcinoma syndrome, also known as Gorlin-Goltz syndrome, is an uncommon multisystem disease with an autosomal dominant trait, as well as a high degree of penetrance and variable expressivity. It is characterized by a wide range of developmental abnormalities and a predisposition to neoplastic change. The main clinical manifestations include numerous basal cell nevi, odontogenic keratocysts of the jaws, musculoskeletal abnormalities, intracranial ectopic calcifications, as well as facial dimorphism, and ophthalmologic abnormalities. Clinical diagnosis of this disease relies on certain criteria. Early detection of the syndrome is very vital  as the disease becomes more destructive with age apart from its high susceptibility to neoplastic transformation. Management requires a multidisciplinary approach. Surgical removal is the mainstay of treatment.