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DeCS
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Descriptor English:
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Basal Cell Nevus Syndrome
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Descriptor Spanish:
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Síndrome del Nevo Basocelular
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Descriptor Portuguese:
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Síndrome do Nevo Basocelular
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Synonyms English:
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Fifth Phacomatoses
Fifth Phacomatosis
Gorlin Goltz Syndrome
Gorlin Syndrome
Gorlin-Goltz Syndrome
Multiple Basal Cell Nevi, Odontogenic Keratocysts, and Skeletal Anomalies
NBCCS
Nevoid Basal Cell Carcinoma Syndrome
Nevus Syndrome, Basal Cell
Syndrome, Gorlin
Syndrome, Gorlin-Goltz
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Tree Number:
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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
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Definition English:
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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. |
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Indexing Annotation English:
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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
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History Note English:
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91(80); was see under CARCINOMA, BASAL CELL 1980-90
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Allowable Qualifiers English:
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Record Number:
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23952
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Unique Identifier:
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D001478
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Occurrence in VHL:
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Similar:
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DeCS
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