Nevoid basal cell carcinoma syndrome: a review of the literature

Management of multiple odontogenic keratocyst in a case of Gorlin-Goltz syndrome and literature review

“…Three major criteria: Histologically proven odontogenic keratocyst of jaw, palmar pits, and calcification of falx cerebri; minor criteria, i.e., skeletal anomalies such as frontal bossing, bifid ribs, mild Multiple odontogenic keratocysts are the first as well as the most consistent and representative sign in this syndrome with an incidence of 75% and mandible being the most common site. [3][4][5][6][7] There may be remarkably few symptoms until cyst reach a large size, especially when in the ascending ramus which supports our case who was asymptomatic at presentation and multiple cystic lesions were noted on routine radiographic examination. Microscopically, they are multilocular with a parakeratinized stratified squamous epithelium most common type (96%) which is identical to our case.…”

“…[5] Diagnosis is made with the presence of two major criteria, 1 major criteria and molecular confirmation or 1 major and 2 minor criteria. [2][3][4][5] The age predilection for this condition is in the 1-3 rd decade of life supporting our case who presented to us in the second decade of life. [5] Although in our case, the patient did not present with basal cell carcinoma, there were other major and minor criteria fulfilling the diagnosis of the syndrome.…”

“…Pathogenesis is attributed to loss of human patched gene (PTCH1 gene) which is a tumor suppressor gene linked to the long arm of chromosome 9 (q22.3-q31). [3] The diagnosis is based on the 1991 Evans et al diagnostic criteria which were later modified by Kimonis et al [4] in 1997 and Bree et al in 2011 [ Table 1]. [5] Diagnosis is made with the presence of two major criteria, 1 major criteria and molecular confirmation or 1 major and 2 minor criteria.…”

Gorlin’s syndrome — Report of a case and management of cystic lesions

“…It is characterized by cutaneous basal cell carcinomas, multiple keratocysts in the jaw bones and skeletal anomalies [16]. It affects male and female equally and seen during the first, second and third decades of life [6,8].…”

Le syndrome de Gorlin-Goltz : rapport d’un cas

“…Cette fréquence le place en troisième position des kystes odontogéniques derrière le kyste radiculaire et le kyste dentigère selon la classi cation de l'OMS [14]. Lorsqu'il se présente de façon isolée -sans association avec le syndrome -, il survient principalement au cours des deuxièmes et troisièmes décennies [15,16], ce qui est le cas chez le patient présenté. En revanche, chez les patients atteints du syndrome de Gorlin-Goltz, les kératokystes apparaissent plus tôt, pendant la première décennie, ils représentent souvent la première manifestation de la maladie [17].…”