title: Syndrome in question: Gorlin-Goltz syndrome.
abstract: The Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an uncommon disorder caused by a mutation in Patched, tumor suppressor gene. It is mainly characterized by numerous early onset basal cell carcinomas, odontogenic cysts of jaw and skeletal abnormalities. Due to the wide clinical spectrum, treatment and management of its modalities are not standardized and should be individualized and monitored by a multidisciplinary team. We report a typical case in a 30-year-old man with multiple basal cell carcinomas, keratotic pits of palmar creases and bifid ribs, with a history of several corrective surgeries for keratocystic odontogenic tumors, among other lesions characteristic of the syndrome.
authors: Hospital Santa Casa de Misericórdia de Vitória (HSCMV) - Vitória (ES), Brazil.
authors: Pauline Lyrio
authors: PL
authors: Ribeiro
authors: Hospital Santa Casa de Misericórdia de Vitória (HSCMV) - Vitória (ES), Brazil.
authors: João Basílio de
authors: JB
authors: Souza
authors: Hospital Santa Casa de Misericórdia de Vitória (HSCMV) - Vitória (ES), Brazil.
authors: Karina Demoner de
authors: KD
authors: Abreu
authors: Hospital Santa Casa de Misericórdia de Vitória (HSCMV) - Vitória (ES), Brazil.
authors: Marisa Simon
authors: MS
authors: Brezinscki
authors: Hospital Santa Casa de Misericórdia de Vitória (HSCMV) - Vitória (ES), Brazil.
authors: Christine Chambo
authors: CC
authors: Pignaton
mesh_terms: D000328:Adult; D001478:Basal Cell Nevus Syndrome / Q000473:pathology*; D006801:Humans; D006972:Hypertelorism / Q000473:pathology; D008297:Male; D009807:Odontogenic Cysts / Q000000981:diagnostic imaging / Q000473:pathology; D011862:Radiography, Panoramic; D012600:Scoliosis / Q000000981:diagnostic imaging / Q000473:pathology; D012878:Skin Neoplasms / Q000473:pathology*