Disease · MONDO_0018271

peripheral primitive neuroectodermal tumor

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. peripheral primitive neuroectodermal tumor. Diseases, record MONDO_0018271. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0018271

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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: peripheral primitive neuroectodermal tumor; Open Targets
Open Targets ID: MONDO_0018271; Open Targets
Other names: PPNET, peripheral PNET, peripheral neuroectodermal neoplasm, peripheral neuroectodermal tumor, peripheral neuroectodermal tumour, peripheral neuroepithelioma, peripheral primitive neuroectodermal neoplasm; Open Targets
NCI Thesaurus: C9341; NCI Thesaurus
Orphanet: ORPHA:370348; Orphanet
Highest stage of its drugs: Phase 2, for 8 of its 13 drugs, from ClinicalTrials.gov; Open Targets

Drugs13

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
CIXUTUMUMAB	CHEMBL1743001	Antibody	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
ETOPOSIDE	CHEMBL44657	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
IFOSFAMIDE	CHEMBL1024	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
VINCRISTINE	CHEMBL90555	Small molecule	Phase 2	ClinicalTrials.gov	2	2	2	Drugs and targets
DOXORUBICIN	CHEMBL53463	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
IXABEPILONE	CHEMBL1201752	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
PEMETREXED	CHEMBL225072	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
BUSULFAN	CHEMBL820	Small molecule	Phase 1	ClinicalTrials.gov	1	1	1	Drugs and targets
DOXORUBICIN HYDROCHLORIDE	CHEMBL359744	Small molecule	Phase 1	ClinicalTrials.gov	1	1	1	Drugs and targets

1 to 10 of 13

See all 13 drugs

Targets22

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 2	https://conductscience.org/library/genes/7153	Drugs and targets
TUBA1A	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/7846	Drugs and targets
TUBA1B	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/10376	Drugs and targets
TUBA1C	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/84790	Drugs and targets
TUBA3C	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/7278	Drugs and targets
TUBA3E	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/112714	Drugs and targets
TUBA4A	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/7277	Drugs and targets
TUBB	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/203068	Drugs and targets
TUBB1	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/81027	Drugs and targets
TUBB2A	Tubulin	2	Tubulin inhibitor	Phase 2	https://conductscience.org/library/genes/7280	Drugs and targets

1 to 10 of 22

See all 22 targets

Trials7

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
NCT00668148	A Five-Tier, Open-Label Study of IMC-A12 in Advanced Sarcoma	Phase 2	113	2018-07-17	Ewing's Sarcoma /Peripheral Neuroectodermal Tumor (PNET), Rhabdomyosarcoma, Leiomyosarcoma, Adipocytic Sarcoma, Synovial Sarcoma	CIXUTUMUMAB	Its record names the disease	ClinicalTrials.gov
NCT00520936	A Study of Pemetrexed in Children With Recurrent Cancer	Phase 2	72	2011-02-23	Osteosarcoma, Medulloblastoma, Sarcoma, Ewing's, Neuroblastoma (Measurable Disease), Neuroblastoma (Metaiodobenzylguanidine, Positive Evaluable), Rhabdomyosarcoma, Ependymoma, Non-brainstem High-grade Glioma	PEMETREXED	Open Targets' reading of its record	ClinicalTrials.gov
NCT00001209	A Pilot Study for the Treatment of Patients With Metastatic and High Risk Sarcomas and Primitive Neuroectodermal Tumors	Phase 1	120		Neuroectodermal Tumor, Primitive, Neuroepithelioma, Osteosarcoma, Sarcoma, Sarcoma, Ewing's	CYCLOPHOSPHAMIDE, DOXORUBICIN HYDROCHLORIDE, ETOPOSIDE, IFOSFAMIDE, VINCRISTINE	Its record names the disease	ClinicalTrials.gov
NCT00331643	Ixabepilone in Treating Young Patients With Refractory Solid Tumors	Phase 2	120		Adult Rhabdomyosarcoma, Adult Synovial Sarcoma, Alveolar Childhood Rhabdomyosarcoma, Childhood Synovial Sarcoma, Embryonal Childhood Rhabdomyosarcoma, Previously Treated Childhood Rhabdomyosarcoma, Recurrent Adult Soft Tissue Sarcoma, Recurrent Childhood Rhabdomyosarcoma, Recurrent Childhood Soft Tissue Sarcoma, Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor, Recurrent Neuroblastoma, Recurrent Osteosarcoma, Recurrent Wilms Tumor and Other Childhood Kidney Tumors	IXABEPILONE	Its record names the disease	ClinicalTrials.gov
NCT00609141	IMC-A12 in Treating Young Patients With Relapsed or Refractory Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor or Other Solid Tumor	Phase 1	34		Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor, Unspecified Childhood Solid Tumor, Protocol Specific	CIXUTUMUMAB	Its record names the disease	ClinicalTrials.gov
NCT00003081	Combination Chemotherapy, Peripheral Stem Cell Transplantation, and Radiation Therapy in Treating Patients With Ewing's Sarcoma, Peripheral Primitive Neuroectodermal Tumor, or Rhabdomyosarcoma	Phase 1	16		Sarcoma	BUSULFAN, FILGRASTIM, MELPHALAN, THIOTEPA	Its record names the disease	ClinicalTrials.gov
NCT00002466	Combination Chemotherapy and Radiation Therapy in Treating Patients With Peripheral Neuroectodermal Tumors, Ewing's Sarcoma, Wilms' Tumor, or Bone Cancer	Phase 2			Kidney Cancer, Sarcoma	CYCLOPHOSPHAMIDE, DOXORUBICIN, ETOPOSIDE, IFOSFAMIDE, VINCRISTINE	Open Targets' reading of its record	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 trials4

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
10797354	RESULT	1	PubMed
8622067	RESULT	1	PubMed
7595741	RESULT	1	PubMed
22682017	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_0018271

GET https://conductscience.org/api/diseases/v1/MONDO_0018271/targets?page=2

API reference For AI assistants