Squamous cell carcinoma of the maxillary sinus associated with nevoid basal cell carcinoma syndrome: report of a case with 21-year evaluation.

摘要

The first recognition of nevoid basal cell carcinoma syndrome (NBCCS) occurred in I960 by 2 scientists, Gorlin and Goltz.1 It is an autosomal dominant syndrome distinguished by various signs and symptoms. The major diagnostic features include multiple keratocystic odontogenic tumors (KCOTs) of the maxilla and mandible, multiple cutaneous basal cell carcinomas, skeletal abnormalities, palmar pits, calcification of the falx cerebri, parietal and temporal bossing, and mental retardation.