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Basal Cell Nevus Syndrome

Hereditary disorder consisting of multiple basal cell carcinomas, odontogenic keratocysts, and multiple skeletal defects, e.g., frontal and temporoparietal bossing, bifurcated and splayed ribs, kyphoscoliosis, fusion of vertebrae, and cervicothoracic spina bifida. Genetic transmission is autosomal dominant.

Year introduced: 1991(1980)

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Tree Number(s): C04.182.089.530.690.150, C04.557.470.200.165.150, C04.557.470.565.165.150, C04.700.175, C05.116.099.105, C05.500.470.690.150, C07.320.450.670.130, C16.131.077.130, C16.320.700.175

MeSH Unique ID: D001478

Entry Terms:

  • Gorlin Syndrome
  • Syndrome, Gorlin
  • Gorlin-Goltz Syndrome
  • Gorlin Goltz Syndrome
  • Syndrome, Gorlin-Goltz
  • NBCCS
  • Nevoid Basal Cell Carcinoma Syndrome
  • Nevus Syndrome, Basal Cell
  • Fifth Phacomatosis
  • Fifth Phacomatoses
  • Multiple Basal Cell Nevi, Odontogenic Keratocysts, and Skeletal Anomalies

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