Kiran N K, Tilak Raj T N, Mukunda K S, Rajashekar Reddy V
Department of Pedodontics and Preventive Dentistry, Sri Siddhartha Dental College and Hospital, Agalakote, Tumkur, Karnataka, India.
Contemp Clin Dent. 2012 Oct;3(4):514-8. doi: 10.4103/0976-237X.107459.
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 odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.
戈林-戈尔茨综合征，也称为痣样基底细胞癌综合征（NBCCS），是一种罕见的多系统疾病，以常染色体显性方式遗传，具有高外显率和可变表达性。其特征为颌骨牙源性角化囊肿、多发性基底细胞痣癌和骨骼异常。由于牙源性角化囊肿通常是该综合征的首批表现之一，牙医可在患者生命的第一个十年通过常规影像学检查对该综合征进行早期诊断。本病例报告介绍了一名13岁男孩，通过临床、影像学和组织学检查结果被诊断为NBCCS。本文强调了早期诊断NBCCS的重要性，这有助于采取预防性多学科方法，为患者提供更好的预后。
