The naevoid basal cell carcinoma syndrome. Clinical, biochemical and radiological aspects.

Eight living patients with the nevoid basal cell carcinoma syndrome (NBS) underwent a clinical and radiological examination, and data on a deceased patient was included. Cerebral CT-scannings were performed on eight patients, and calcification in the falx and tentorium cerebelli was found in all patients but one. Congenital hydrocephalus or agenesis of the corpus callosum could not be demonstrated. Laboratory results, including serum levels of alpha 1-antitrypsin and alpha 2-macroglobulin, haptoglobin, inorganic phosphorus, alkaline phosphatase and calcium, were normal in all patients but two. It is postulated that elevated serum alkaline phosphatase in patients with NBS is correlated to the presence of growing odontogenic keratocysts.