The Nevoid Basal Cell Carcinoma Syndrome

Three of our patients with the nevoid basal cell carcinoma syndrome died as a result of their disease. The clinical and autopsy findings on these patients and others recorded in the literature are the subject of this report. Some of the unusual autopsy findings included direct extension of the basal cell carcinoma into the brain in two cases with metastasis to other parts of the brain and lung in one case, congenital anomalies of the brain in three cases, kidney anomalies in two cases, leiomyomas in three cases, adrenal cortical adenomas in two cases, fibrocystic disease of the breast in two of the three women, and oxyphil cell nodular hyperplasia of the parathyroid gland in one case.