Familial multiple odontogenic keratocysts.
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.
Wang
Xu-Xia
XX
Department of Oral and Maxillofacial Surgery, Shandong University School of Stomatology, Jinan, Shandong, People's Republic of China.
Zhang
Jun
J
Wei
Feng-Cai
FC
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
D007633:Keratins