Korean J Otolaryngol-Head Neck Surg.  2007 Jun;50(6):552-555.

A Case of Basal Cell Nevus Syndrome with Odontogenic Keratocyst

Affiliations
  • 1Department of Otorhinolaryngology-Head and Neck Surgery, Sungkyunkwan University, Samsung Medical Center, Seoul, Korea. hysiam.kim@samsung.com

Abstract

Nevoid basal cell carcinoma syndrome (Gorlin syndrome) is a cancer predisposition syndrome characterized by multiple basal cell carcinomas and diverse developmental defects. Recently, we experienced a case of odontogenic keratocysts in a 13-year-old boy who was finally diagnosed as having nevoid basal cell carcinoma syndrome by genetic study. The purpose of this paper is to report odontogenic keratocyst as the first manifestation of nevoid basal cell carcinoma syndrome and to emphasize that one must consider this syndrome as a possible diagnosis in all cases of odontogenic keratocysts.

Keyword

Nevoid basal cell carcinoma syndrome; Odontogenic keratocyst; Gorlin syndrome

MeSH Terms

Adolescent
Basal Cell Nevus Syndrome*
Carcinoma, Basal Cell
Diagnosis
Humans
Male
Odontogenic Cysts*
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