Table 2.

Diagnostic criteria for nevoid basal-cell carcinoma syndrome based on the consensus statement from the first international colloquium on nevoid basal-cell carcinoma syndrome[7]

Diagnostic criteria for NBCCS

Major criteria

BCC before 20 years old or excessive numbers of  BCC out of proportion to prior sun exposure and skin type  OKCs of the jaw before 20 years of age  Palmar or plantar pitting  Lamellar calcification of the falx cerebri  MED  First-degree relative with NBCCS

Minor criteria

Rib anomalies  Skeletal malformations and radiologic changes (i.e., vertebral anomalies, kyphoscoliosis, short fourth metacarpals, and postaxial polydactyly)  Macrocephaly  Cleft lip/palate  Ovarian/cardiac fibroma  Lymphomesentric cysts  Ocular abnormalities (i.e., strabismus, hypertelorism, congenital cataracts, glaucoma, and coloboma)

BCC: Basal-cell carcinoma, OKC: Odontogenic keratocysts, NBCCS: Nevoid basal-cell carcinoma syndrome, MED: Medulloblastoma