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peripheral primitive neuroectodermal tumor (PPNET) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

peripheral primitive neuroectodermal tumor

MONDO:0018271

Also known as:PPNETperipheral PNETperipheral neuroectodermal neoplasm

Find Clinical TrialsTrials

Overview

A small round cell tumor with neural differentiation arising from the soft tissues or bone.

Clinical Trials14 registered · 2 recruiting

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include always present findings: Peripheral primitive neuroectodermal neoplasm; and sometimes findings: Torticollis, Jaundice, Pruritus, and Seizure and others. 37 total HPO annotations.

Organ System	Phenotype Count	Example Features
Digestive system	7	Jaundice, Ascites, Pancreatitis

Diagnosis

Biomarker and diagnostic research for peripheral primitive neuroectodermal tumor has been reported in the published literature.

Prognosis

Phenotype severity distribution: 1 always present feature.

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

14 clinical trials registered, 2 recruiting. Interventions under study include drug therapy, procedural interventions, other interventions, and biologic therapy. Pipeline includes 1 PHASE3, 10 PHASE2, 2 PHASE1. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT04901702](https://clinicaltrials.gov/study/NCT04901702)

Data assembled from 5 of 12 sources · Last updated Sep 20, 2026, 1:11 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:370348

GARD

Genetic and Rare Diseases Info Center

GARD:17601

Questions for Your Care Team

→Are any of the 14 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Research Type	Count	% of Total
Patient case studies	11	69%
Research summaries	3	19%
Testing and diagnosis research	1	6%
Clinical study results	1	6%

Kisho

peripheral primitive neuroectodermal tumor

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications

peripheral primitive neuroectodermal tumor

Overview

Clinical Presentation

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Recent Publications