Article ID Journal Published Year Pages File Type
3122071 Asian Journal of Oral and Maxillofacial Surgery 2011 8 Pages PDF
Abstract

In the present report, we review the clinical features and biophysiological roles, including gene expression, of nevoid basal cell carcinoma syndrome (NBCCS) in Japanese cases. The frequency of clinical findings of Japanese cases (n = 186) was reported in NBCCS including keratocystic odontogenic tumors (KOTs). The syndromes resulted from mutation in the patched (PTCH1) gene in both neoplastic lesion basal cell carcinoma (BCCs) and KOTs. BCCs originate from hair follicles and express Gli gene; KOTs, classically known as odontogenic keratocysts, originate from odontogenic epithelial rest cells and considerable variation in the recurrence rate after surgery. PTCH1 gene alteration may have significant roles in the pathogenesis of NBCCS and sporadic KOTs. However, their histogenetic origin was found to differ: basal cell carcinoma in the skin originated from hair follicle cells and KOTs originated from odontogenic epithelial rest cells in the jaw.

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