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embryonal tumor with multilayered rosettes - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

embryonal tumor with multilayered rosettes

MONDO:0958119

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Overview

A rare central nervous system embryonal tumor characterized by embryonal cells arranged in multilayered rosettes and displaying one of three morphological patterns: embryonal tumor with abundant neuropil and true rosettes, ependymoblastoma, or medulloepithelioma. The tumors typically have a C19MC alteration or (rarely) a DICER1 mutation and correspond to WHO grade IV. They are mostly localized intracranially, rarely in the spinal cord, and commonly cause signs and symptoms of elevated intracranial pressure, sometimes seizures and focal neurological signs. Most cases occur in children during the first two years of life.

Clinical Trials11 registered · 10 recruiting

Diagnosis

Biomarker and diagnostic research for embryonal tumor with multilayered rosettes has been reported in the published literature.

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.

11 clinical trials registered, 10 recruiting. Interventions under study include drug therapy, biologic therapy, other interventions, and procedural interventions. Pipeline includes 1 PHASE2, 7 PHASE1, 1 EARLY_PHASE1. Research is primarily sponsored by academic and government institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT05278208](https://clinicaltrials.gov/study/NCT05278208)

Data assembled from 4 of 12 sources · Last updated Sep 18, 2026, 9:11 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:656417

GARD

Genetic and Rare Diseases Info Center

GARD:6352

Questions for Your Care Team

→Are any of the 11 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?

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Research Type	Count	% of Total
Laboratory research	9	38%
Patient case studies	7	29%
Testing and diagnosis research	2	8%
Research summaries	2	8%
Clinical study results	2	8%
Disease patterns and progression	1	4%
New treatment approaches	1	4%

embryonal tumor with multilayered rosettes

Overview

Diagnosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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