Table 1.

Diagnostic criteria for nevoid basal cell carcinoma [Gorlin] syndrome.

Diagnosis of NBCCS requires two major or one major and two minor criteria

Major criteria

More than two basal cell carcinomas, or one basal cell carcinoma in a patient younger than 20 y

Odontogenic keratocysts of the jaw bone

Three or more palmar or plantar pits

Calcification of the falx cerebri

Bifid, fused, or markedly splayed ribs

First-degree relative with NBCCS

Minor criteria

Ovarian fibroma

Medulloblastoma

Macrocephaly

Craniofacial malformations: cleft lip or palate, frontal bossing, coarse facial features, hypertelorism

Skeletal abnormalities: Sprengel deformity, pectus deformity, syndactyly

Radiologic abnormalities: bridging of the sella turcica, vertebral anomalies (i.e., hemivertebrae, fusion or elongation of the vertebral bodies), modeling defects of the hands and feet (i.e., flame-shaped lucencies of the hands or feet)

Note: NBCCS = nevoid basal cell carcinoma [Gorlin] syndrome.