Early diagnosis of Gorlin-Goltz syndrome: case report

The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by keratocystic odontogenic tumors (KCOT) in the jaw, multiple basal cell nevi carcinomas and skeletal abnormities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the KCOTs are usually one of the first manifestations of the syndrome. This article paper reports the case of a patient, a 10-year-old boy with NBCCS, emphasizing its clinical and radiographic manifestations. This study highlights the importance of health professionals in the early diagnosis of NBCCS and in a preventive multidisciplinary approach to provide a better prognosis for the patient.

The authors are grateful to Patrícia FreitasFaria (Bauru School of Dentistry, University of São Paulo) and thank her for the valuable technical support with images. The authors also wish to thank the patient and their family for their contribution to this article. Written consent for publication was obtained from the patients parent.

The authors are grateful to Patrícia Freitas-Faria (Bauru School of Dentistry, University of São Paulo) and thank her for the valuable technical support with images. The authors also wish to thank the patient and their family for their contribution to this article. Written consent for publication was obtained from the patient's parent.