ABSTRACT
The nevoid basal cell carcinoma syndrome (NBCCS) is characterized by numerous basal cell cancers and epidermal cysts of the skin, odontogenic keratocysts, palmoplantar pits, calcified dural folds, various neoplasms or hamartomas, which include ovarian fibromas, medulloblastoma, lymphomesenteric cysts, fetal rhabdomyomas, etc., and various stigmata of maldevelopment (rib and vertebral abnormalities, cleft lip and/or palate, cortical defects of bones, etc. Hermans et al., 1965) (Box 64.1, 64.2).
