J Korean Assoc Maxillofac Plast Reconstr Surg.  2008 Mar;30(2):172-177.

Case report of multiple odontogenic keratocysts with basal cell nevus syndrome

• Park JH
• Yoon KH
• Park KS
• Cheong JK
• Shin JM
• Choi MK

Abstract

Basal cell nevus syndrome, also known as Gorlin-Goltz syndrome, is a hereditary condition transmitted as an autosomal dominant trait that exhibits high penetrance and variable expressivity. It is an ecto-mesodermal polydysplasia with numerous manifestations that affect multiple organs. Odontogenic keratocysts, palmar and plantar pits, and hypertelorism are the most frequently observed anomalies. Currently there are new lines of investigation based on biomolecular studies, which aim at identifying the molecules responsible for these cysts and thus early allowing an early diagnosis of these patients. We report two cases of multiple odontogenic keratocysts associated with basal cell nevus syndrome.

Keyword

Odontogenic keratocyst; Basal cell nevus syndrome

MeSH Terms

Basal Cell Nevus Syndrome
Early Diagnosis
Humans
Hypertelorism
Odontogenic Cysts
Penetrance