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Descriptor English: Basal Cell Nevus Syndrome
Descriptor Spanish: Síndrome del Nevo Basocelular
Descriptor síndrome del nevo basocelular
Entry term(s) síndrome de Gorlin
síndrome de Gorlin Goltz
síndrome de Gorlin-Goltz
síndrome del carcinoma basocelular nevoide
síndrome del nevus basocelular
Scope note: Enfermedad hereditaria que consiste en múltiples carcinomas basocelulares, queratoquistes odontógenos y defectos esqueléticos múltiples, como protuberancias frontales y temporoparietales, costillas bifurcadas y abiertas, cifoescoliosis, fusión de vértebras y espina bífida cervicotorácica. La transmisión genética es autosómica dominante.
Descriptor Portuguese: Síndrome do Nevo Basocelular
Descriptor French: Naevomatose basocellulaire
Entry term(s): 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
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
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D001478
Scope note: 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.
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
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
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)
Public MeSH Note: 91; was see under CARCINOMA, BASAL CELL 1980-90
History Note: 91(80); was see under CARCINOMA, BASAL CELL 1980-90
DeCS ID: 23952
Unique ID: D001478
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1991/01/01
Date of Entry: 1979/04/25
Revision Date: 2013/07/08
Basal Cell Nevus Syndrome - Preferred
Concept UI M0002195
Scope note 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.
Preferred term Basal Cell Nevus Syndrome
Entry term(s) 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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