Nevoid basal cell carcinoma syndrome: a retrospective analysis of 33 affected Korean individuals

• Published in: International journal of oral and maxillofacial surgery Vol. 33; no. 5; pp. 458 - 462
• Main Authors: Ahn, S.-G, Lim, Y.-S, Kim, D.-K, Kim, S.-G, Lee, S.-H, Yoon, J.-H
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier Ltd 01.07.2004; Elsevier
• Subjects: Adolescent; Adult; Age Factors; Basal Cell Nevus Syndrome - epidemiology; Biological and medical sciences; Calcinosis - epidemiology; Central Nervous System Diseases - epidemiology; Child; Dentistry; Dermatology; Dura Mater - pathology; Female; Foot Deformities - epidemiology; Hand Deformities - epidemiology; Humans; Hypertelorism - epidemiology; Incidence; Korea - epidemiology; Korean; Male; Medical sciences; Middle Aged; nevoid basal cell carcinoma syndrome; Odontogenic Cysts - epidemiology; odontogenic keratocyst; Otorhinolaryngology. Stomatology; Retrospective Studies; Tumors of the skin and soft tissue. Premalignant lesions; Asia; South Korea; Korea; odontogenic keratocyst; Korean; nevoid basal cell carcinoma syndrome; Human; Skin disease; Treatment; Stomatology; Surgery; Odontogenic cyst; Basal cell nevus syndrome; Retrospective; Genetic disease
• ISSN: 0901-5027; 1399-0020
• DOI: 10.1016/j.ijom.2003.11.001

This article describes a pooled analysis of Korean individuals with nevoid basal cell carcinoma syndrome (NBCCS). The data upon which this review is based has been retrieved from published case reports in Korean dental and medical literature between the years 1981 to 2002. We found 33 subjects who met the diagnostic criteria for NBCCS. Relative frequencies of associated complications are presented and compared with those of the English literature. Odontogenic keratocyst (OKC) and palmar and/or plantar pits, and hypertelorism were the most frequently observed anomalies. OKCs are often the first signs of NBCCS and can be detected in patients younger than 20 years of age. However, the incidence and clinical manifestations of NBCCS in Korean individuals were found to be rather different from those of other countries. The relatively low frequency of basal cell carcinomas and falx calcification among the major criteria were two major differences. The frequencies of the minor criteria concur in general with the ranges given by some others. It is concluded that these differences may be attributed to genetic and geographic differences.