Case Report
Odontogenic keratocysts in Nevoid basal cell carcinoma syndrome: a case report
-
* Corresponding author: Masume Johari dr_masume@yahoo.com
1 Department of Oral and Maxillofacial Pathology, Faculty of Dentistry and Dental Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
2 Department of Oral and Maxillofacial Radiology, Faculty of Dentistry and Dental Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
3 Department of Oral and Maxillofacial Pathology, Faculty of Dentistry and Dental Research Center, Hamedan University of Medical Sciences, Hamedan, Iran
4 Department of Oral and Maxillofacial surgery, Faculty of Dentistry and Dental Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
5 Department of Oral and Maxillofacial Radiology, Faculty of Dentistry and Dental Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
Cases Journal 2009, 2:9399 doi:10.1186/1757-1626-2-9399
Published: 24 December 2009Abstract
Nevoid basal cell carcinoma syndrome, a rare autosomal dominant disorder, comprises a number of abnormalities such as multiple nevoid basal cell carcinomas, skeletal abnormalities and multiple odontogenic keratocysts. Considering the rarity of this syndrome, we present a 12-year-old boy affected by this syndrome. He had multiple okcs, calcification of falx cerebri, bifid ribs, frontal bossing and hypertelorism. Characteristic cutaneous manifestation (nevoid basal cell carcinoma) was not present in this patient. The jaw cysts were treated with marsupialization then enucleation. The dental clinician may be the first to encounter and identify this syndrome, when the multiple cystlike radiolucencies are discovered on panoramic view.