Commentary: The Nevoid Basal Cell Carcinoma Syndrome

Abstract

One of the most interesting, stimulating, and important genodermatoses to come to light during the 20th century is the nevoid basal cell carcinoma (NBCC) (basal cell nevus)¹syndrome. The multisystem aspects of this disorder have stimulated interest and research in a variety of medical and basic science specialties. The termbasal cell nevuswas coined by Nomland²when he reported an unusual case of invasive basal cell carcinoma of the face that occurred in adult life from congenital pigmented basal cell tumors. Clinically, the tumors resembled pigmented moles (nevi); however, microscopically, the cells were "like dark staining basal cells." Thus, the name "nevus of basal cells" was used. The original report that defined this syndrome described only three major components: basal cell nevi, jaw cysts, and rib defects.¹We now know that, in its fullest expression, the syndrome comprises the following five major components3: multiple nevoid basal