title: Familial multiple odontogenic keratocysts.
abstract: Multiple odontogenic keratocysts (OKCs) are principle features of nevoid basal cell carcinoma syndrome (NBCCS; Gorlin-Goltz syndrome). NBCCS is a genetic disorder transmitted by an autosomal dominant gene with variable expressivity, which is important to recognize when a patient has multiple OKCs. The cysts of the jaws are among the most common findings. Another feature is a certain appearance of the face, such as: large calvaria, high-arched eyebrows, broad nasal root, and mild hypertelorism. Before-therapy diagnosis is, therefore, as important as after-therapy diagnosis. Genetic counseling and examination may also be indicated. The purpose of this paper was to present a family case report of nevoid basal cell carcinoma syndrome with multiple odontogenic keratocysts. The features identified by these combined clinical, imaging, and histologic findings are described, along with a brief mention of the family history and a review of the literature.
authors.affiliation: Department of Oral and Maxillofacial Surgery, Shandong University School of Stomatology, Jinan, Shandong, People's Republic of China.
authors.forename: Xu-Xia
authors.initials: XX
authors.lastname: Wang
authors.forename: Jun
authors.initials: J
authors.lastname: Zhang
authors.forename: Feng-Cai
authors.initials: FC
authors.lastname: Wei
mesh_terms: D000293:Adolescent; D000328:Adult; D001478:Basal Cell Nevus Syndrome / Q000150:complications*; D019066:Facies; D005192:Family Health; D005260:Female; D006801:Humans; D007570:Jaw Cysts / Q000150:complications* / Q000000981:diagnostic imaging; D007633:Keratins; D008297:Male; D014057:Tomography, X-Ray Computed
chemical_list: D007633:Keratins