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.

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

Instance information

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

identifier

D001478