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Odontogenic keratocysts in Nevoid basal cell carcinoma syndrome: a case report

Authors
Journal
Cases Journal
1757-1626
Publisher
Springer (Biomed Central Ltd.)
Publication Date
Volume
2
Issue
1
Identifiers
DOI: 10.1186/1757-1626-2-9399
Keywords
  • Case Report

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.

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