NBCCS

nevoid basal cell carcinoma syndrome(English)

  • an autosomal dominant tumor-predisposing disorder characterized by two hallmark features: jaw cysts (odontogenic keratocysts) and multiple cutaneous basal cell carcinomas. Other features include skeletal anomalies, large head size and an increased risk of developing childhood-onset medulloblastoma and other types of cancer. Awareness among ophthalmic specialists is important as periocular basal cell carcinomas may be the presenting feature in a minority of cases. Children of these patients have a 50% chance of inheriting the condition. Careful clinical examination with a skeletal survey can usually diagnose the syndrome by teenage years. Aka Gorlin syndrome
  • SUFU, MB
  • Dermatology, Oncology, Dentistry, Skeleton and bone (formation), Genetics, Genomics
  • https://en.wiki…cinoma_syndrome
  • https://doi.org…13944-8.00023-8