Major Criteria
■ Multiple (>2) basal cell carcinomas at any age or one basal cell carcinoma less than 30 years or >10 basal cell naevi
■ Histologically proven odontogenic keratocyst or a polyostotic bone cyst
■ Palmar or plantar pits (3 or more)
■ Ectopic calcification: lamellar or early (<20 years) calcification of the falx cerebri
■ Family history of Gorlin's syndrome
Minor Criteria
■ Congenital skeletal defects: bifid, fused, splayed, or missing rib, or bifid, wedged, or fused vertebra
■ Large head with occipitofrontal circumference >97th percentile, with frontal bossing
■ Cardiac or ovarian fibroma (benign tumour in heart or ovary)
■ Medulloblastoma (a malignant brain tumour that usually arises in young children)
■ Lymphomesenteric cysts (abdominal cysts full of lymph fluid)
■ Congenital malformation: cleft lip and/or palate, polydactyly (extra fingers or toes), congenital eye defect such as cataract, microphthalmos (small eye) or coloboma (iris tumour)
Table 1: Major and minor criteria for the diagnosis of NBCCS.