The multiple nevoid basal cell carcinoma syndrome revisited

Abstract

Over a decade has elapsed since the senior author became interested in the multiple nevoid basal cell carcinoma syndrome. During the intervening years many facets of the syndrome have been examined and the evidence weighed concerning new findings. The original triad of signs, multiple nevoid basal cell carcinomas, jaw cysts and skeletal anomalies, has been greatly expanded to include intracranial calcification, ovarian fibroma, lymphomesenteric cysts, medulloblastoma and a whole host of minor and occasional (but valid) associated anomalies.