The multiple basal-cell nevi syndrome.An analysis of a syndrome consisting of multiple nevoid basal-cell carcinoma, jaw cysts, skeletal anomalies, medulloblastoma, and hyporesponsiveness to parathormone

Abstract

T cell carcinoma, odontogenic keratocysts, and skeletal anomalies is far more complex and encompassing than was supposed when first described. It involves many organs but principally affects the skin and the endocrine, skeletal, and nervous systems. Because of the publication of several of these cases in 1960 and 1963,339 34 one of the authors ( R . J . G . ) has had numerous communications from individuals telling of their cases and leading us to others heretofore untabulated. Within the past few years, additional information has been added, implicating other body systems. Our present survey would suggest that there are at least 38 reports of this syndrome, involving more than 150 individuals. I n addition, at least 10 reports of "familial multiple dentigerous cysts" involving 37 individuals were available for analysis. Moreover, there were 28 reports of single or nonfamilial cases of multiple jaw cysts. T h e relationship between these entities and the complete syndrome was therefore deemed worthy of investigation.