Disease · MONDO_0000640

central nervous system primitive neuroectodermal neoplasm

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. central nervous system primitive neuroectodermal neoplasm. Diseases, record MONDO_0000640. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0000640

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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: central nervous system primitive neuroectodermal neoplasm; Open Targets
Open Targets ID: MONDO_0000640; Open Targets
Other names: CNS PNET, CNS primitive neuroectodermal neoplasm, CNS primitive neuroectodermal tumor, CNS primitive neuroectodermal tumour, central nervous system PNET, central nervous system neuroectodermal tumor, central nervous system neuroectodermal tumour, central nervous system primitive neuroectodermal tumor, central nervous system primitive neuroectodermal tumor (WHO grade IV), central nervous system primitive neuroectodermal tumour, central nervous system primitive neuroectodermal tumour (WHO grade IV), central primitive neuroectodermal neoplasm, central primitive neuroectodermal tumor, central primitive neuroectodermal tumour, primitive neuroectodermal tumor of central nervous system, primitive neuroectodermal tumour of central nervous system; Open Targets
Disease Ontology: DOID:0060103; Disease Ontology
NCI Thesaurus: C5398; NCI Thesaurus
Highest stage of its drugs: Phase 3, for 4 of its 8 drugs, from ClinicalTrials.gov; Open Targets

Drugs8

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.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
CARBOPLATIN	CHEMBL1351	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
ETOPOSIDE	CHEMBL44657	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
VINCRISTINE	CHEMBL90555	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
ADAVOSERTIB	CHEMBL1976040	Small molecule	Phase 1/2	ClinicalTrials.gov	1	1	1	Drugs and targets
DECITABINE	CHEMBL1201129	Small molecule	Phase 1/2	ClinicalTrials.gov	1	1	1	Drugs and targets
HILTONOL	CHEMBL4297559		Phase 1/2	ClinicalTrials.gov	1	1	1	Drugs and targets
IRINOTECAN HYDROCHLORIDE	CHEMBL3989514	Small molecule	Phase 1/2	ClinicalTrials.gov	1	1	1	Drugs and targets

Targets21

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.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
DNMT1	DNA (cytosine-5)-methyltransferase 1	1	DNA (cytosine-5)-methyltransferase 1 inhibitor	Phase 1/2	https://conductscience.org/library/genes/1786	Drugs and targets
DNMT3A	DNA (cytosine-5)-methyltransferase 3A	1	DNA (cytosine-5)-methyltransferase 3A inhibitor	Phase 1/2	https://conductscience.org/library/genes/1788	Drugs and targets
TOP1	DNA topoisomerase 1	1	DNA topoisomerase I inhibitor	Phase 1/2	https://conductscience.org/library/genes/7150	Drugs and targets
TOP2A	DNA topoisomerase II	1	DNA topoisomerase II inhibitor	Phase 3	https://conductscience.org/library/genes/7153	Drugs and targets
TOP2B	DNA topoisomerase II	1	DNA topoisomerase II inhibitor	Phase 3	https://conductscience.org/library/genes/7155	Drugs and targets
TUBA1A	Tubulin	1	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/7846	Drugs and targets
TUBA1B	Tubulin	1	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/10376	Drugs and targets
TUBA1C	Tubulin	1	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/84790	Drugs and targets
TUBA3C	Tubulin	1	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/7278	Drugs and targets
TUBA3E	Tubulin	1	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/112714	Drugs and targets

1 to 10 of 21

See all 21 targets

Trials3

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.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT02095132	Adavosertib and Irinotecan Hydrochloride in Treating Younger Patients With Relapsed or Refractory Solid Tumors	Phase 1/2	76	2022-10-05	Central Nervous System Embryonal Tumor With Rhabdoid Features, Central Nervous System Embryonal Tumor, Not Otherwise Specified, Central Nervous System Ganglioneuroblastoma, Embryonal Tumor With Multilayered Rosettes, C19MC-Altered, Pineoblastoma, Primary Central Nervous System Neoplasm, Recurrent Childhood Central Nervous System Embryonal Neoplasm, Recurrent Malignant Solid Neoplasm, Recurrent Medulloblastoma, Recurrent Neuroblastoma, Recurrent Rhabdomyosarcoma, Refractory Malignant Solid Neoplasm, Refractory Medulloblastoma, Refractory Neuroblastoma, Refractory Rhabdomyosarcoma	ADAVOSERTIB, IRINOTECAN HYDROCHLORIDE	Its record names the disease	ClinicalTrials.gov
NCT02332889	Phase I/II: Decitabine/Vaccine Therapy in Relapsed/Refractory Pediatric High Grade Gliomas/Medulloblastomas/CNS PNETs	Phase 1/2	1	2018-04-30	Gliomas, Medulloblastoma, Neuroectodermal Tumors, Primitive	DECITABINE, HILTONOL	Open Targets' reading of its record	ClinicalTrials.gov
NCT00003859	Surgery Plus Radiation Therapy With or Without Chemotherapy in Treating Children With Primitive Neuroectodermal Tumors of the CNS	Phase 3	230		Brain and Central Nervous System Tumors	CARBOPLATIN, CYCLOPHOSPHAMIDE, ETOPOSIDE, VINCRISTINE	Its record names the disease	ClinicalTrials.gov

Comparisons in its trials0

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.

None in this release.

Papers from its trials7

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

PMID	Listed as	Trials listing it	Source
19328574	RESULT	1	PubMed
17878477	RESULT	1	PubMed
16258095	RESULT	1	PubMed
15328187	RESULT	1	PubMed
15001263	RESULT	1	PubMed
12697884	RESULT	1	PubMed
34171339	DERIVED	1	PubMed

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_0000640

GET https://conductscience.org/api/diseases/v1/MONDO_0000640/targets?page=2

API reference For AI assistants