Disease · MONDO_0005462

primitive neuroectodermal tumor

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. primitive neuroectodermal tumor. Diseases, record MONDO_0005462. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0005462

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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: primitive neuroectodermal tumor; Open Targets
Open Targets ID: MONDO_0005462; Open Targets
Other names: PNET, neuroectodermal neoplasm, neuroectodermal tumor, neuroectodermal tumour, primitive neuroectodermal neoplasm, primitive neuroectodermal tumor (PNET), primitive neuroectodermal tumour (PNET); Open Targets
Disease Ontology: DOID:171; Disease Ontology
MeSH: D017599; MeSH
NCI Thesaurus: C3716; NCI Thesaurus
Highest stage of its drugs: Phase 3, for 14 of its 35 drugs, from ClinicalTrials.gov; Open Targets

Drugs35

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
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 3	ClinicalTrials.gov	13	13	13	Drugs and targets
ETOPOSIDE	CHEMBL44657	Small molecule	Phase 3	ClinicalTrials.gov	11	11	11	Drugs and targets
CARBOPLATIN	CHEMBL1351	Small molecule	Phase 3	ClinicalTrials.gov	8	8	8	Drugs and targets
FILGRASTIM	CHEMBL1201567	Protein	Phase 3	ClinicalTrials.gov	8	8	8	Drugs and targets
CISPLATIN	CHEMBL11359	Small molecule	Phase 3	ClinicalTrials.gov	7	7	7	Drugs and targets
VINCRISTINE	CHEMBL90555	Small molecule	Phase 3	ClinicalTrials.gov	7	7	7	Drugs and targets
METHOTREXATE	CHEMBL34259	Small molecule	Phase 3	ClinicalTrials.gov	6	6	6	Drugs and targets
THIOTEPA	CHEMBL671	Small molecule	Phase 3	ClinicalTrials.gov	6	6	6	Drugs and targets
TEMOZOLOMIDE	CHEMBL810	Small molecule	Phase 3	ClinicalTrials.gov	4	4	4	Drugs and targets
LEUCOVORIN CALCIUM	CHEMBL1201138	Small molecule	Phase 3	ClinicalTrials.gov	3	3	3	Drugs and targets

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Targets44

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
TOP2A	DNA topoisomerase 2-alpha	3	DNA topoisomerase II alpha inhibitor; DNA topoisomerase II inhibitor	Phase 3	https://conductscience.org/library/genes/7153	Drugs and targets
EGFR	Epidermal growth factor receptor	2	Epidermal growth factor receptor erbB1 inhibitor	Phase 1/2	https://conductscience.org/library/genes/1956	Drugs and targets
ERBB2	Receptor tyrosine-protein kinase erbB-2	2	Receptor protein-tyrosine kinase erbB-2 inhibitor	Phase 1/2	https://conductscience.org/library/genes/2064	Drugs and targets
TOP1	DNA topoisomerase 1	2	DNA topoisomerase I inhibitor	Phase 2	https://conductscience.org/library/genes/7150	Drugs and targets
TUBA1A	Tubulin	2	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/7846	Drugs and targets
TUBA1B	Tubulin	2	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/10376	Drugs and targets
TUBA1C	Tubulin	2	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/84790	Drugs and targets
TUBA3C	Tubulin	2	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/7278	Drugs and targets
TUBA3E	Tubulin	2	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/112714	Drugs and targets
TUBA4A	Tubulin	2	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/7277	Drugs and targets

1 to 10 of 44

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Trials27

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. 1 more is not shown, since the checks do not confirm it.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT00392327	Chemotherapy and Radiation Therapy in Treating Young Patients With Newly Diagnosed, Previously Untreated, High-Risk Medulloblastoma/PNET	Phase 3	379	2020-08-07	Anaplastic Medulloblastoma, Medulloblastoma	CARBOPLATIN, CISPLATIN, CYCLOPHOSPHAMIDE, ISOTRETINOIN, VINCRISTINE SULFATE	Its record names the disease	ClinicalTrials.gov
NCT01154816	Alisertib in Treating Young Patients With Recurrent or Refractory Solid Tumors or Leukemia	Phase 2	118	2017-06-05	Hepatoblastoma, Previously Treated Childhood Rhabdomyosarcoma, Recurrent Childhood Acute Lymphoblastic Leukemia, Recurrent Childhood Acute Myeloid Leukemia, Recurrent Childhood Kidney Neoplasm, Recurrent Childhood Malignant Germ Cell Tumor, Recurrent Childhood Rhabdomyosarcoma, Recurrent Childhood Soft Tissue Sarcoma, Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor, Recurrent Neuroblastoma, Recurrent Osteosarcoma	ALISERTIB	Its record names the disease	ClinicalTrials.gov
NCT01217437	Temozolomide and Irinotecan Hydrochloride With or Without Bevacizumab in Treating Young Patients With Recurrent or Refractory Medulloblastoma or CNS Primitive Neuroectodermal Tumors	Phase 2	108	2019-09-13	Central Nervous System Neoplasm, Pineoblastoma, Recurrent Medulloblastoma, Recurrent Primitive Neuroectodermal Tumor, Refractory Medulloblastoma, Refractory Peripheral Primitive Neuroectodermal Tumor	BEVACIZUMAB, IRINOTECAN HYDROCHLORIDE, TEMOZOLOMIDE	Its record names the disease	ClinicalTrials.gov
NCT00357500	Etoposide, Cyclophosphamide, Thalidomide, Celecoxib, and Fenofibrate in Relapsed or Progressive Cancer	Phase 2	101	2014-09-09	Central Nervous System Tumor, Pediatric, Leukemia, Lymphoma, Neuroblastoma, Sarcoma, Unspecified Childhood Solid Tumor, Protocol Specific	CELECOXIB, CYCLOPHOSPHAMIDE, ETOPOSIDE, FENOFIBRATE, THALIDOMIDE	Its record names the disease	ClinicalTrials.gov
NCT00336024	Combination Chemotherapy Followed By Peripheral Stem Cell Transplant in Treating Young Patients With Newly Diagnosed Supratentorial Primitive Neuroectodermal Tumors or High-Risk Medulloblastoma	Phase 3	91	2018-10-24	Anaplastic Medulloblastoma, Medulloblastoma, Supratentorial Embryonal Tumor, Not Otherwise Specified, Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor	CISPLATIN, CYCLOPHOSPHAMIDE, ETOPOSIDE, FILGRASTIM, LEUCOVORIN CALCIUM, METHOTREXATE, VINCRISTINE	Its record names the disease	ClinicalTrials.gov
NCT02372006	Trial of Afatinib in Pediatric Tumours	Phase 1/2	56	2021-03-04	Neuroectodermal Tumors, Rhabdomyosarcoma	AFATINIB	Open Targets' reading of its record	ClinicalTrials.gov
NCT00095940	Lapatinib in Treating Young Patients With Recurrent or Refractory Central Nervous System Tumors	Phase 1/2	52	2012-02-06	Recurrent Childhood Anaplastic Astrocytoma, Recurrent Childhood Brain Stem Glioma, Recurrent Childhood Ependymoma, Recurrent Childhood Giant Cell Glioblastoma, Recurrent Childhood Glioblastoma, Recurrent Childhood Gliosarcoma, Recurrent Childhood Medulloblastoma, Recurrent Childhood Oligodendroglioma	LAPATINIB	Its record names the disease	ClinicalTrials.gov
NCT00003460	Antineoplaston Therapy in Treating Children With Primitive Neuroectodermal Tumors	Phase 2	13	2016-11-10	Childhood CNS Primitive Neuroectodermal Tumor	ANTINEOPLASTON A10	Its record names the disease	ClinicalTrials.gov
NCT00006734	Comparison of Combination Chemotherapy Regimens in Treating Patients With Ewing's Sarcoma or Neuroectodermal Tumor	Phase 3	587		Sarcoma	CYCLOPHOSPHAMIDE, DOXORUBICIN HYDROCHLORIDE, ETOPOSIDE, FILGRASTIM, IFOSFAMIDE, VINCRISTINE SULFATE	Its record names the disease	ClinicalTrials.gov
NCT00749723	Therapy Optimization Trial for the Treatment of Relapsed or Refractory Brain Tumors in Children	Phase 2/3	174		Recurrent Brain Tumors, Supratentorial PNETs, Medulloblastomas, Ependymomas	CARBOPLATIN, ETOPOSIDE, TEMOZOLOMIDE, THIOTEPA, TROFOSFAMIDE	Its record names the disease	ClinicalTrials.gov

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Comparisons in its trials11

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.

Comparison	Group 1, with the outcome	Group 2, with the outcome	Participants	Trial	Source
Arm I (Temozolomide, Irinotecan Hydrochloride) vs Arm II (Temozolomide, Irinotecan Hydrochloride, Bevacizumab): deaths from any cause	48 of 54	42 of 52	106	NCT01217437	Trial comparisons
Arm I (Temozolomide, Irinotecan Hydrochloride) vs Arm II (Temozolomide, Irinotecan Hydrochloride, Bevacizumab): serious adverse events	9 of 54	12 of 52	106	NCT01217437	Trial comparisons
Arm I (Patients Treated Without Methotrexate (MTX)) vs Arm II (Patients Treated With MTX): Number of Participants With Chronic Primary Hypothyroidism/Subclinical Compensatory HypothyroidismHypothyroidism/Subclinical Compensatory Hypothyroidism	3 of 39	5 of 38	77	NCT00336024	Trial comparisons
Arm I (Patients Treated Without Methotrexate (MTX)) vs Arm II (Patients Treated With MTX): Number of Participants With Chronic Low Somatomedin C	2 of 39	5 of 38	77	NCT00336024	Trial comparisons
Arm I (Patients Treated Without Methotrexate (MTX)) vs Arm II (Patients Treated With MTX): Number of Participants With Secondary Malignancies	1 of 39	0 of 38	77	NCT00336024	Trial comparisons
Arm I (Patients Treated Without Methotrexate (MTX)) vs Arm II (Patients Treated With MTX): Number of Participants With Chronic Diabetes Insipidus	1 of 39	1 of 38	77	NCT00336024	Trial comparisons
Arm I (Patients Treated Without Methotrexate (MTX)) vs Arm II (Patients Treated With MTX): deaths from any cause	18 of 39	15 of 38	77	NCT00336024	Trial comparisons
Arm I (Patients Treated Without Methotrexate (MTX)) vs Arm II (Patients Treated With MTX): Number of Participants With Chronic Central Hypothyroidism	1 of 39	1 of 38	77	NCT00336024	Trial comparisons
Arm I (Patients Treated Without Methotrexate (MTX)) vs Arm II (Patients Treated With MTX): serious adverse events	8 of 39	3 of 38	77	NCT00336024	Trial comparisons
Dose Finding - Level 0 vs Dose Finding - Level 1: Number of Participants With Dose Limiting Toxicity Adverse Events - Dose Finding Part	1 of 8	2 of 9	17	NCT02372006	Trial comparisons

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Papers from its trials15

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
30332335	RESULT	1	PubMed
23091096	RESULT	1	PubMed
21480204	RESULT	1	PubMed
18447671	RESULT	1	PubMed
16408203	RESULT	1	PubMed
16382125	RESULT	1	PubMed
9849694	RESULT	1	PubMed
9771955	RESULT	1	PubMed
37651654	DERIVED	1	PubMed
37178647	DERIVED	1	PubMed

1 to 10 of 15

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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_0005462

GET https://conductscience.org/api/diseases/v1/MONDO_0005462/drugs?page=2

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