Table 1.

Diagnostic criteria for nevoid basal cell carcinoma syndrome (adapted from [23])

Major criteria

1. Basal cell carcinoma before 20 years of age or excessive numbers of basal cell carcinomas out of proportion to prior sun exposure and skin type

2. Keratocystic odontogenic tumor before 20 years of age

3. Palmar or plantar pitting

4. Lamellar calcification of the falx cerebri

5. Medulloblastoma, typically desmoplastic

6. First degree relative with nevoid basal cell carcinoma syndrome

Minor criteria

1. Rib abnormalities

2. Other specific skeletal malformations and radiologic changes (i.e., vertebral anomalies, kyphoscoliosis, short fourth metacarpals, postaxial polydactyly)

3. Macrocephaly

4. Cleft lip or palate

5. Ovarian or cardiac fibroma

6. Lymphomesenteric cysts

7. Ocular abnormalities (i.e., strabismus, hypertelorism, congenital cataracts, glaucoma, coloboma)