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Basal Cell Nevus Syndrome
MeSH HeadingBasal Cell Nevus Syndrome
Tree NumberC04.182.089.530.690.150
Tree NumberC04.557.470.200.165.150
Tree NumberC04.557.470.565.165.150
Tree NumberC04.700.175
Tree NumberC05.116.099.105
Tree NumberC05.500.470.690.150
Tree NumberC07.320.450.670.130
Tree NumberC16.131.077.130
Tree NumberC16.320.700.175
Annotationcoordinate 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
Scope NoteHereditary 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.
Entry TermFifth Phacomatosis
Entry TermGorlin Syndrome
Entry TermGorlin-Goltz Syndrome
Entry TermMultiple Basal Cell Nevi, Odontogenic Keratocysts, and Skeletal Anomalies
Entry TermNBCCS
Entry TermNevoid Basal Cell Carcinoma Syndrome
Entry TermNevus Syndrome, Basal Cell
Allowable QualifiersBL CF CI CL CO DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RA RH RI RT SU TH UR US VE VI
Previous Indexing Bone Cyst (1966-1979)
Previous Indexing Carcinoma, Basal Cell (1966-1979)
Previous Indexing Jaw Diseases (1967-1979)
Previous Indexing Jaw Neoplasms (1967-1979)
Previous Indexing Skin Neoplasms (1967-1979)
Previous Indexingspecific jaw disease (1967-1979)
Previous Indexingspecific jaw neoplasm (1967-1979)
Previous Indexing Syndrome (1972-1979)
History Note91(80); was see under CARCINOMA, BASAL CELL 1980-90
Date of Entry19790425
Unique IDD001478


MeSH Tree Structures

 
Neoplasms [C04]
   Cysts [C04.182]
      Bone Cysts [C04.182.089]
         Jaw Cysts [C04.182.089.530]
            Odontogenic Cysts [C04.182.089.530.690]
Arrow pointing to current tree nodeBasal Cell Nevus Syndrome [C04.182.089.530.690.150]
Dentigerous Cyst [C04.182.089.530.690.310]
Odontogenic Cyst, Calcifying [C04.182.089.530.690.605]
Periodontal Cyst [C04.182.089.530.690.790]  +

 
Neoplasms [C04]
   Neoplasms by Histologic Type [C04.557]
      Neoplasms, Glandular and Epithelial [C04.557.470]
         Carcinoma [C04.557.470.200]
            Carcinoma, Basal Cell [C04.557.470.200.165]
Arrow pointing to current tree nodeBasal Cell Nevus Syndrome [C04.557.470.200.165.150]

 
Neoplasms [C04]
   Neoplasms by Histologic Type [C04.557]
      Neoplasms, Glandular and Epithelial [C04.557.470]
         Neoplasms, Basal Cell [C04.557.470.565]
            Carcinoma, Basal Cell [C04.557.470.565.165]
Arrow pointing to current tree nodeBasal Cell Nevus Syndrome [C04.557.470.565.165.150]

 
Neoplasms [C04]
   Neoplastic Syndromes, Hereditary [C04.700]
Adenomatous Polyposis Coli [C04.700.100]  +
Arrow pointing to current tree nodeBasal Cell Nevus Syndrome [C04.700.175]
Birt-Hogg-Dube Syndrome [C04.700.212]
Colorectal Neoplasms, Hereditary Nonpolyposis [C04.700.250]  +
Dysplastic Nevus Syndrome [C04.700.305]
Exostoses, Multiple Hereditary [C04.700.330]
Hamartoma Syndrome, Multiple [C04.700.435]
Hereditary Breast and Ovarian Cancer Syndrome [C04.700.517]
Li-Fraumeni Syndrome [C04.700.600]
Multiple Endocrine Neoplasia [C04.700.630]  +
Tuberous Sclerosis [C04.700.632]
Wilms Tumor [C04.700.635]  +
Neurofibromatoses [C04.700.645]  +
Peutz-Jeghers Syndrome [C04.700.705]

 
Musculoskeletal Diseases [C05]
   Bone Diseases [C05.116]
      Bone Diseases, Developmental [C05.116.099]
Acro-Osteolysis [C05.116.099.052]  +
Arrow pointing to current tree nodeBasal Cell Nevus Syndrome [C05.116.099.105]
Dwarfism [C05.116.099.343]  +
Dysostoses [C05.116.099.370]  +
Funnel Chest [C05.116.099.386]
Gigantism [C05.116.099.492]
Leg Length Inequality [C05.116.099.655]
Marfan Syndrome [C05.116.099.674]
Osteochondrodysplasias [C05.116.099.708]  +
Osteolysis, Essential [C05.116.099.736]
Platybasia [C05.116.099.742]
Proteus Syndrome [C05.116.099.750]
Weill-Marchesani Syndrome [C05.116.099.875]

 
Musculoskeletal Diseases [C05]
   Jaw Diseases [C05.500]
      Jaw Cysts [C05.500.470]
         Odontogenic Cysts [C05.500.470.690]
Arrow pointing to current tree nodeBasal Cell Nevus Syndrome [C05.500.470.690.150]
Dentigerous Cyst [C05.500.470.690.310]
Odontogenic Cyst, Calcifying [C05.500.470.690.605]
Periodontal Cyst [C05.500.470.690.790]  +

 
Stomatognathic Diseases [C07]
   Jaw Diseases [C07.320]
      Jaw Cysts [C07.320.450]
         Odontogenic Cysts [C07.320.450.670]
Arrow pointing to current tree nodeBasal Cell Nevus Syndrome [C07.320.450.670.130]
Dentigerous Cyst [C07.320.450.670.275]
Odontogenic Cyst, Calcifying [C07.320.450.670.495]
Periodontal Cyst [C07.320.450.670.513]  +

 
Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
   Congenital Abnormalities [C16.131]
      Abnormalities, Multiple [C16.131.077]
22q11 Deletion Syndrome [C16.131.077.019]  +
Alagille Syndrome [C16.131.077.065]
Alstrom Syndrome [C16.131.077.080]
Angelman Syndrome [C16.131.077.095]
Bardet-Biedl Syndrome [C16.131.077.112]
Barth Syndrome [C16.131.077.121]
Arrow pointing to current tree nodeBasal Cell Nevus Syndrome [C16.131.077.130]
Beckwith-Wiedemann Syndrome [C16.131.077.133]
Bloom Syndrome [C16.131.077.137]
Branchio-Oto-Renal Syndrome [C16.131.077.208]
Carney Complex [C16.131.077.229]
CHARGE Syndrome [C16.131.077.239]
Cockayne Syndrome [C16.131.077.250]
Costello Syndrome [C16.131.077.256]
Cri-du-Chat Syndrome [C16.131.077.262]
De Lange Syndrome [C16.131.077.272]
Deaf-Blind Disorders [C16.131.077.299]  +
Donohue Syndrome [C16.131.077.313]
Down Syndrome [C16.131.077.327]
Ectodermal Dysplasia [C16.131.077.350]  +
Fraser Syndrome [C16.131.077.371]
Gardner Syndrome [C16.131.077.393]
Heterotaxy Syndrome [C16.131.077.401]
Holoprosencephaly [C16.131.077.410]
Incontinentia Pigmenti [C16.131.077.445]
Isolated Noncompaction of the Ventricular Myocardium [C16.131.077.477]
Laurence-Moon Syndrome [C16.131.077.509]
LEOPARD Syndrome [C16.131.077.525]
Loeys-Dietz Syndrome [C16.131.077.537]
Marfan Syndrome [C16.131.077.550]
Mobius Syndrome [C16.131.077.578]
Monilethrix [C16.131.077.592]
Nail-Patella Syndrome [C16.131.077.606]
Netherton Syndrome [C16.131.077.619]
Nevus, Sebaceous of Jadassohn [C16.131.077.633]
Oculocerebrorenal Syndrome [C16.131.077.661]
Orofaciodigital Syndromes [C16.131.077.677]
Pallister-Hall Syndrome [C16.131.077.690]
Pentalogy of Cantrell [C16.131.077.696]
POEMS Syndrome [C16.131.077.703]
Prader-Willi Syndrome [C16.131.077.730]
Prolidase Deficiency [C16.131.077.735]
Proteus Syndrome [C16.131.077.740]
Prune Belly Syndrome [C16.131.077.745]
Rubella Syndrome, Congenital [C16.131.077.790]
Rubinstein-Taybi Syndrome [C16.131.077.804]
Short Rib-Polydactyly Syndrome [C16.131.077.850]
Silver-Russell Syndrome [C16.131.077.855]
Smith-Lemli-Opitz Syndrome [C16.131.077.860]
Smith-Magenis Syndrome [C16.131.077.879]
Sotos Syndrome [C16.131.077.889]
Trichothiodystrophy Syndromes [C16.131.077.899]
Waardenburg Syndrome [C16.131.077.938]
Weill-Marchesani Syndrome [C16.131.077.941]
Wolf-Hirschhorn Syndrome [C16.131.077.944]
Wolfram Syndrome [C16.131.077.951]
Zellweger Syndrome [C16.131.077.970]

 
Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
   Genetic Diseases, Inborn [C16.320]
      Neoplastic Syndromes, Hereditary [C16.320.700]
Adenomatous Polyposis Coli [C16.320.700.100]  +
Arrow pointing to current tree nodeBasal Cell Nevus Syndrome [C16.320.700.175]
Birt-Hogg-Dube Syndrome [C16.320.700.212]
Colorectal Neoplasms, Hereditary Nonpolyposis [C16.320.700.250]  +
Dysplastic Nevus Syndrome [C16.320.700.305]
Exostoses, Multiple Hereditary [C16.320.700.330]
Hamartoma Syndrome, Multiple [C16.320.700.435]
Hereditary Breast and Ovarian Cancer Syndrome [C16.320.700.517]
Li-Fraumeni Syndrome [C16.320.700.600]
Multiple Endocrine Neoplasia [C16.320.700.630]  +
Tuberous Sclerosis [C16.320.700.636]
Wilms Tumor [C16.320.700.642]  +
Neurofibromatoses [C16.320.700.645]  +
Peutz-Jeghers Syndrome [C16.320.700.705]

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