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.

Also Known As:

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

Neoplasms: 1361496
- Cysts: 26232
  - Bone Cysts: 885
    - Jaw Cysts: 140
      - Odontogenic Cysts: 695
        Basal Cell Nevus Syndrome: 362
- Hereditary Neoplastic Syndromes: 115
  - Basal Cell Nevus Syndrome: 362
- Neoplasms by Histologic Type: 2
  - Glandular and Epithelial Neoplasms: 445
    - Carcinoma: 143226
      - Basal Cell Carcinoma: 4796
        Basal Cell Nevus Syndrome: 362
    - Basal Cell Neoplasms: 67
      - Basal Cell Carcinoma: 4796
        Basal Cell Nevus Syndrome: 362
Musculoskeletal Diseases: 751
- Bone Diseases: 5970
  - Developmental Bone Diseases: 156
    - Basal Cell Nevus Syndrome: 362
- Jaw Diseases: 15
  - Jaw Cysts: 140
    - Odontogenic Cysts: 695
      - Basal Cell Nevus Syndrome: 362
Stomatognathic Diseases: 725
- Jaw Diseases: 15
  - Jaw Cysts: 140
    - Odontogenic Cysts: 695
      - Basal Cell Nevus Syndrome: 362
Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 960
- Congenital Abnormalities: 26404
  - Multiple Abnormalities: 213
    - Basal Cell Nevus Syndrome: 362
- Inborn Genetic Diseases: 12342
  - Hereditary Neoplastic Syndromes: 115
    - Basal Cell Nevus Syndrome: 362

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