Nevoid Basal Cell Carcinoma Syndrome

Abstract

MIDFACIAL cysts were the subject of a recent article by Beekhuis and Watson.¹ A classification of odontogenic and nonodontogenic cysts was given with discussion of the embryology and significance to the otolaryngologist. The association of odontogenic cysts with multiple basal cell carcinoma skin lesions and skeletal anomalies was described in 1951 by Binkley and Johnson.² Multiple basal cell carcinoma skin lesions are now known to be associated on rare occasions with developmental anomalies of the skeletal, cutaneous, endocrine, ophthalmic, and central nervous systems. These findings have been collectively termed the basal cell nevus or nevoid basal cell carcinoma syndrome. Since the first report by Binkley and Johnson, many reports^3-8 of this syndrome have reached the general medical and dental literature. As yet, this syndrome has not been reported in the otolaryngologic literature and this fact prompted the present article. As in the case herein reported, odontogenic