Diseases

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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.

MeSH Record

Classification

Broader headings

Related Concepts

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MeSH Record

Synonyms

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

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

Indexing Annotation

coordinate IM with precoordinated organ/neoplasm (IM or NIM) only if pertinent; do not confuse entry term GORLIN-GOLTZ SYNDROME with GOLTZ- GORLIN SYNDROME see FOCAL DERMAL HYPOPLASIA

MeSH Record

History Note

91(80); was see under CARCINOMA, BASAL CELL 1980-90

MeSH Record

Previous Indexing

  • Bone Cyst (1966-1979)
  • Carcinoma, Basal Cell (1966-1979)
  • Jaw Diseases (1967-1979)
  • Jaw Neoplasms (1967-1979)
  • Skin Neoplasms (1967-1979)
  • Syndrome (1972-1979)
  • specific jaw disease (1967-1979)
  • specific jaw neoplasm (1967-1979)

MeSH Hierarchy

Tree Numbers

  1. C04w.182w.089w.530w.690w.150w
  2. C04w.557.470.200w.649.002w.001w
  3. C04w.588.805.511.002w.001w
  4. C04w.700w.175w
  5. C05w.116w.099.105w
  6. C05w.500.470w.690w.150w
  7. C07.320.450w.670w.130w
  8. C16w.131w.077w.130w
  9. C16w.320w.700w.175w
  10. C17.800w.882.623.002w.001w

AMA Style

References

  1. National Library of Medicine. Basal Cell Nevus Syndrome. Medical Subject Headings (MeSH). 2026. Unique ID D001478. http://id.nlm.nih.gov/mesh/2026/D001478
  2. Basal Cell Nevus Syndrome. In: Wikipedia. https://en.wikipedia.org/wiki/Nevoid_basal-cell_carcinoma_syndrome
  3. Basal Cell Nevus Syndrome. In: Wikidata. https://www.wikidata.org/wiki/Q1536720