Abstract
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.
Highlights
Nevoid basal cell carcinoma syndrome (NBCCS), is an autosomal dominant disorder with a high degree of penetrance and a variable expressivity characterized by several developmental defects and predisposition to cancer
Considering the rarity of this syndrome we present a young subject affected by NBCCS
NBCCS is a hereditary complex of the abnormalities transmitted as an autosomal dominant trait
Summary
Nevoid basal cell carcinoma syndrome (NBCCS), is an autosomal dominant disorder with a high degree of penetrance and a variable expressivity characterized by several developmental defects and predisposition to cancer. The syndrome, first delineated by Gorlin and Goltz, is characterized by basal cell carcinoma, odontogenic keratocysts, palmar and/or plant pits and ectopic calcification of the falx cerebri [2,3,4]. These traits are considered major clinical diagnostic criteria. Seventy-five percent of patients affected by NBCCS often show multiple and bilateral odontogenic keratocysts They are mainly located in the premolar area, may displace teeth with consequent malocclusion and can be unilocular or multilocular with a preference for the mandible. The panoramic radiography depicted two large radiolucent lesions With these findings, we suspected multiple jaw cysts associated with NBCCS. One year after surgery, there was no evidence of recurrence of jaw cysts
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
