Multiple, Multiloculated, and Recurrent Keratocysts of the Mandible and Maxilla in Association with Gorlin-Goltz (Nevoid Basal-Cell Carcinoma) Syndrome: A Pediatric Case Report and Follow-up over 5 Years
Table 1
Diagnostic criteria for nevoid basal-cell carcinoma syndrome. A diagnosis can be made when two major or one major and two minor criteria are fulfilled. Extracted from Evans et al. [14].
Major criteria
Minor criteria
More than two basal cell carcinomas, or BCCs; one BCC at younger than 30 years of age; or more than 10 basal cell nevi
Congenital skeletal anomaly: bifid, fused, splayed or missing rib or bifid, wedged or fused vertebra
Any odontogenic keratocyst (proven on histology) or polyostotic bone cyst
Occipital-frontal circumference, more than 97 percentile, with frontal bossing
Three or more palmar or plantar pits
Cardiac or ovarian fibroma
Ectopic calcification: lamellar or early—at younger than 20 years of age—falx calcification
Medulloblastoma
Positive family history of nevoid basal-cell carcinoma syndrome
Lymphomesenteric cysts
Congenital malformation such as cleft lip or palate, polydactylism, or eye anomaly (cataract, coloboma, microphthalmos)