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Disease · MONDO_0007187

nevoid basal cell carcinoma syndrome

6 sourcesdiseases-2026-09-18-0.1.0

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ConductScience Library. nevoid basal cell carcinoma syndrome. Diseases, record MONDO_0007187. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0007187

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Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name
nevoid basal cell carcinoma syndrome
Open Targets
Open Targets ID
MONDO_0007187
Open Targets
Other names
Gorlin syndrome, Gorlin-Goltz syndrome, NBCCS, basal cell nevus syndrome, multiple basal cell carcinomas, nevoid basal cell cancer syndrome
Open Targets
Disease Ontology
DOID:0070365
Disease Ontology
Disease Ontology
DOID:2512
Disease Ontology
MeSH
D001478
MeSH
NCI Thesaurus
C2892
NCI Thesaurus
Orphanet
ORPHA:377
Orphanet
Highest stage of its drugs
Phase 3, for 1 of its 7 drugs, from ClinicalTrials.gov
Open Targets

Drugs7

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

DrugChEMBL IDTypeOpen Targets' stageStage reported byReportsOf them trialsTrials shown hereSource
PATIDEGIBCHEMBL538867Small moleculePhase 3ClinicalTrials.gov433Drugs and targets
VISMODEGIBCHEMBL473417Small moleculePhase 2ClinicalTrials.gov444Drugs and targets
SONIDEGIBCHEMBL2105737Small moleculePhase 2ClinicalTrials.gov333Drugs and targets
NIVOLUMABCHEMBL2108738AntibodyPhase 2ClinicalTrials.gov111Drugs and targets
TAZAROTENECHEMBL1657Small moleculePhase 2ClinicalTrials.gov111Drugs and targets
GUSACITINIBCHEMBL4594275Small moleculePhase 1/2ClinicalTrials.gov111Drugs and targets
BMS-833923CHEMBL3545403Small moleculeUnknownClinicalTrials.gov111Drugs and targets

Targets10

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

TargetProteinIts drugs that act on itMechanismsHighest stage of those drugsGene in GenesSource
SMOProtein smoothened4Smoothened homolog antagonist; Smoothened homolog inhibitorPhase 3https://conductscience.org/library/genes/6608Drugs and targets
JAK1Janus Kinase (JAK)1Janus Kinase (JAK) inhibitorPhase 1/2https://conductscience.org/library/genes/3716Drugs and targets
JAK2Janus Kinase (JAK)1Janus Kinase (JAK) inhibitorPhase 1/2https://conductscience.org/library/genes/3717Drugs and targets
JAK3Janus Kinase (JAK)1Janus Kinase (JAK) inhibitorPhase 1/2https://conductscience.org/library/genes/3718Drugs and targets
PDCD1Programmed cell death protein 11Programmed cell death protein 1 inhibitorPhase 2https://conductscience.org/library/genes/5133Drugs and targets
RARARetinoic acid receptor1Retinoic acid receptor agonistPhase 2https://conductscience.org/library/genes/5914Drugs and targets
RARBRetinoic acid receptor1Retinoic acid receptor agonistPhase 2https://conductscience.org/library/genes/5915Drugs and targets
RARGRetinoic acid receptor1Retinoic acid receptor agonistPhase 2https://conductscience.org/library/genes/5916Drugs and targets
SYKTyrosine-protein kinase SYK1Tyrosine-protein kinase SYK inhibitorPhase 1/2https://conductscience.org/library/genes/6850Drugs and targets
TYK2Janus Kinase (JAK)1Janus Kinase (JAK) inhibitorPhase 1/2https://conductscience.org/library/genes/7297Drugs and targets

Trials12

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

TrialTitlePhaseParticipantsResults first postedConditionsDrugsLinked bySource
NCT01815840A Study of Two Vismodegib Regimens in Participants With Multiple Basal Cell CarcinomasPhase 22292016-08-04Basal Cell CarcinomaVISMODEGIBIts record names the diseaseClinicalTrials.gov
NCT03703310Study of Patidegib Topical Gel, 2%, for the Reduction of Disease Burden of Persistently Developing Basal Cell Carcinomas (BCCs) in Subjects With Basal Cell Nevus Syndrome (Gorlin Syndrome)Phase 31742024-07-03Basal Cell Nevus SyndromePATIDEGIBIts record names the diseaseClinicalTrials.gov
NCT04308395Extension Study of Patidegib Topical Gel, 2% in Subjects With Gorlin Syndrome (Basal Cell Nevus Syndrome)Phase 31082024-09-24Basal Cell Nevus SyndromePATIDEGIBIts record names the diseaseClinicalTrials.gov
NCT00957229To Determine The Efficacy and Safety of GDC-0449 in Patients With Basal Cell Nevus Syndrome (BCNS)Phase 2412021-01-11Basal Cell Nevus Syndrome, Gorlin SyndromeVISMODEGIBIts record names the diseaseClinicalTrials.gov
NCT00489086Topical Tazarotene in Treating Patients With Basal Cell Skin Cancer and Basal Cell Nevus Syndrome on the FacePhase 2362016-02-11Neoplastic SyndromeTAZAROTENEIts record names the diseaseClinicalTrials.gov
NCT00961896A Trial to Evaluate the Safety, Local Tolerability, Pharmacokinetics and Pharmacodynamics of LDE225 on Skin Basal Cell Carcinomas in Gorlin Syndrome PatientsPhase 2182015-09-21Treatment for Basal Cell Carcinomas (BCCs) in Gorlin Syndrome PatientsSONIDEGIBIts record names the diseaseClinicalTrials.gov
NCT02762084Trial of Patidegib Gel 2%, 4%, and Vehicle to Decrease the Number of Surgically Eligible Basal Cell Carcinomas in Gorlin Syndrome PatientsPhase 2172019-07-15Basal Cell Nevus SyndromePATIDEGIBIts record names the diseaseClinicalTrials.gov
NCT01350115Efficacy, Safety and Pharmacokinetics of Oral LDE225 in Treatment of Patients With Nevoid Basal Cell Carcinoma Syndrome (NBCCS)Phase 2102015-10-19Basal Cell Carcinoma, Gorlin Syndrome, Nevoid Basal Cell Carcinoma SyndromeSONIDEGIBIts record names the diseaseClinicalTrials.gov
NCT05463757Oral Hedgehog Inhibitors in the Treatment of Basal Cell Carcinoma in the Netherlands: a Prospective Registration Study80
Basal Cell Carcinoma, Locally Advanced Basal Cell Carcinoma, Metastatic Basal Cell Carcinoma, Gorlin Syndrome, Basal Cell Nevus Syndrome, Carcinoma, Basal Cell, Carcinoma, Basal Cell Tumor, Skin Cancer, Neoplasm of Skin, Neoplasms, Basal Cell
SONIDEGIB, VISMODEGIBIts record names the diseaseClinicalTrials.gov
NCT03208296Study of ASN-002 to Treat Basal Cell Carcinomas (BCCs) in Individuals With Basal Cell Nevus Syndrome (BCNS)Phase 1/224Basal Cell Carcinoma in Basal Cell Nevus SyndromeGUSACITINIBIts record names the diseaseClinicalTrials.gov

1 to 10 of 12

See all 12 trials

Comparisons in its trials8

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

ComparisonGroup 1, with the outcomeGroup 2, with the outcomeParticipantsTrialSource
Vismodegib Intermittent Schedule vs Vismodegib Induction Followed by Intermittent Schedule: serious adverse events24 of 11423 of 113227NCT01815840Trial comparisons
Patidegib Topical Gel, 2%, vs Patidegib Topical Gel, Vehicle: deaths from any cause0 of 870 of 87174NCT03703310Trial comparisons
Patidegib Topical Gel, 2%, vs Patidegib Topical Gel, Vehicle: serious adverse events5 of 877 of 87174NCT03703310Trial comparisons
Patidegib Topical Gel, 2%, vs Patidegib Topical Gel, Vehicle: Percentage of Participants Developing >=2 Facial New BCCs45 of 8355 of 86169NCT03703310Trial comparisons
Patidegib Topical Gel, 2%, vs Patidegib Topical Gel, Vehicle: Percentage of Participants Developing >=1 Facial New BCCs56 of 8365 of 86169NCT03703310Trial comparisons
Sugar Pill vs GDC-0449: deaths from any cause0 of 152 of 4055NCT00957229Trial comparisons
Sugar Pill vs GDC-0449: serious adverse events2 of 1521 of 4055NCT00957229Trial comparisons
Patidegib Gel vs Vehicle Gel: The Number of Participants Reporting New SEBs on the Face From Baseline for the Combined Patidegib Treatment Groups2 of 123 of 517NCT02762084Trial comparisons

Papers from its trials6

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMIDListed asTrials listing itSource
27838224RESULT1PubMed
24623282RESULT1PubMed
24441673RESULT1PubMed
22670904RESULT1PubMed
28325399DERIVED1PubMed
28188086DERIVED1PubMed

Data dates

The date of each source this release read.

Open Targets
2026-09-18
Open Targets
Drug links and their checks, from Drugs and targets
2026-09-18
Drugs and targets
ClinicalTrials.gov, from Trials
2026-11-12
Trials
Comparisons, from Trial comparisons
2026-03-23
Trial comparisons
PubMed, for the checks
2026-11-06
PubMed
MeSH, for the checks
2026-11-10
MeSH

Sources 6

  • ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
  • ChEMBL, through Open TargetsCC BY-SA 3.0
  • ClinicalTrials.govNo license stated
  • NLM's MeSHNLM's terms
  • Open TargetsCC0 1.0
  • PubMedNLM's terms
Each source's terms
  • ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
  • ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
  • ClinicalTrials.govnone stated
  • NLM's MeSHNLM's
  • Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
  • PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0007187

GET https://conductscience.org/api/diseases/v1/MONDO_0007187/drugs