Clinical, radiologic, and histopathologic aspects of 13 cases of nevoid basal cell carcinoma syndrome.

Thirteeen patients with the characteristic signs and symptoms of the nevoid basal cell carcinoma syndrome (NBS) are presented. It is suggested that the chromosomal anomalies sporadically reported have hardly any connection with the syndrome. The jaw cysts removed from the patients with NBS were clinically, radiologically, and histopathologically typical keratocysts with a high occurrence of epithelial islands and/or microcyts in the connective tissue of the capsule. The significantly higher occurrence of proliferative epithelial remnants in the connective tissue of the cyst wall may be an explanation of the high recurrence rate seen in patients with NBS and of the recurrence of some solitary keratocysts in patients without any signs or symptoms of NBS. In a follow-up period of 2 years, 85% of the patients with NBS showed recurrence. One-third of these patients did not show any recurrence at the 1-year follow-up. Therefore, a follow-up period of 2 years or more is mandatory in the care of patients with the nevoid basal cell carcinoma syndrome.