Disease · MONDO_0011014

pleuropulmonary blastoma

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. pleuropulmonary blastoma. Diseases, record MONDO_0011014. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0011014

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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: pleuropulmonary blastoma; Open Targets
Open Targets ID: MONDO_0011014; Open Targets
Other names: childhood pulmonary blastoma, paediatric pulmonary blastoma, pediatric pulmonary blastoma, pleuropulmonary blastoma (morphologic abnormality), pulmonary blastoma of childhood; Open Targets
Disease Ontology: DOID:4769; Disease Ontology
MeSH: C537516; MeSH
NCI Thesaurus: C5669; NCI Thesaurus
Orphanet: ORPHA:64742; Orphanet
Highest stage of its drugs: Phase 3, for 7 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
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
DACTINOMYCIN	CHEMBL1554	Protein	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
DOXORUBICIN	CHEMBL53463	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
IFOSFAMIDE	CHEMBL1024	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
VINCRISTINE	CHEMBL90555	Small molecule	Phase 3	ClinicalTrials.gov	2	2	2	Drugs and targets
DEXRAZOXANE	CHEMBL1738	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
TOPOTECAN	CHEMBL84	Small molecule	Phase 3	ClinicalTrials.gov	1	1	1	Drugs and targets
LORVOTUZUMAB MERTANSINE	CHEMBL1743037	Antibody drug conjugate	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets

Targets19

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	2	DNA topoisomerase II alpha inhibitor; DNA topoisomerase II inhibitor	Phase 3	https://conductscience.org/library/genes/7153	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
TUBB	Tubulin	2	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/203068	Drugs and targets
TUBB1	Tubulin	2	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/81027	Drugs and targets
TUBB2A	Tubulin	2	Tubulin inhibitor	Phase 3	https://conductscience.org/library/genes/7280	Drugs and targets

1 to 10 of 19

See all 19 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
NCT02452554	Lorvotuzumab Mertansine in Treating Younger Patients With Relapsed or Refractory Wilms Tumor, Rhabdomyosarcoma, Neuroblastoma, Pleuropulmonary Blastoma, Malignant Peripheral Nerve Sheath Tumor, or Synovial Sarcoma	Phase 2	62	2019-08-29	Pleuropulmonary Blastoma, Recurrent Malignant Peripheral Nerve Sheath Tumor, Recurrent Neuroblastoma, Recurrent Rhabdomyosarcoma, Recurrent Synovial Sarcoma, Wilms Tumor	LORVOTUZUMAB MERTANSINE	Its record names the disease	ClinicalTrials.gov
NCT01464606	International Pleuropulmonary Blastoma (PPB) Treatment and Biology Registry	Not applicable	156		Pleuropulmonary Blastoma	CYCLOPHOSPHAMIDE, DACTINOMYCIN, DOXORUBICIN, IFOSFAMIDE, VINCRISTINE	Its record names the disease	ClinicalTrials.gov
NCT06647953	Testing a Standardized Approach to Surgery and Chemotherapy for Type I Pleuropulmonary Blastoma or the Addition of an Anti-cancer Drug, Topotecan, to the Usual Treatment for Types II and III Pleuropulmonary Blastoma	Phase 3	110		Pleuropulmonary Blastoma	CYCLOPHOSPHAMIDE, DACTINOMYCIN, DEXRAZOXANE, DOXORUBICIN, IFOSFAMIDE, TOPOTECAN, 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 trials0

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

None in this release.

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_0011014

GET https://conductscience.org/api/diseases/v1/MONDO_0011014/drugs

API reference For AI assistants