title: Gorlin-Goltz syndrome in twin brothers: an unusual occurrence with review of the literature.
abstract: Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is caused by genetic alteration produced by a mutation in the 'patched' tumour suppressor gene, and is inherited in a dominant autosomal way. Although sporadic cases have been found, this syndrome has rarely been reported in twin patients. The syndrome is characterised by a wide range of developmental abnormalities and has a predisposition to neoplasms such as multiple pigmented basal cell carcinomas and keratocysts in jaws; it also has other features such as palmar and/or plantar pits and calcification of falx cerebri. Owing to the critical oral and maxillofacial manifestations of this syndrome, it is important to recognise its characteristics in order to make a diagnosis, and to plot early preventive treatment and establish the right genetic evidence. Based on a combination of imaging, clinical and histopathological findings, we present a diagnosed case of Gorlin-Goltz syndrome in 18-year-old twin brothers. All cystic lesions were enucleated and 1 year follow-up showed no recurrence.
authors: Department of Oral & Maxillofacial Surgery, Government Dental College & Hospital, Ahmedabad, Gujarat, India.
authors: Sonal
authors: S
authors: Anchlia
authors: Department of Oral & Maxillofacial Surgery, Government Dental College & Hospital, Ahmedabad, Gujarat, India.
authors: Siddharth
authors: S
authors: Vyas
authors: Department of Oral & Maxillofacial Pathology, KM Shah Dental College, Ahmedabad, Gujarat, India.
authors: Sumit
authors: S
authors: Bahl
authors: Department of Oral & Maxillofacial Surgery, Government Dental College & Hospital, Ahmedabad, Gujarat, India.
authors: Vipul
authors: V
authors: Nagavadiya
mesh_terms: D000293:Adolescent; D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis*; D002114:Calcinosis / Q000000981:diagnostic imaging*; D006801:Humans; D021621:Imaging, Three-Dimensional; D008297:Male; D011859:Radiography; D012886:Skull / Q000000981:diagnostic imaging*; D016135:Spinal Dysraphism / Q000000981:diagnostic imaging*; D014427:Twins