Embryonal tumor with multilayered rosettes

Summary

Embryonal tumor with multilayered rosettes (MONDO:0958119) is a cancer and 8 clinical trials. Top therapeutic interventions include eflornithine, etoposide phosphate, and sonidegib. A subtype of central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Clinical trials: 8

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	embryonal tumor with multilayered rosettes
Mondo ID	MONDO:0958119
Orphanet	656417
NCIT	C186534
UMLS	C5575350
MedGen	1804732
GARD	0006352
Is cancer (heuristic)	yes

Disease family

This is a subtype of central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › nervous system cancer › central nervous system cancer › central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor › embryonal tumor with multilayered rosettes

Related subtypes (3): medulloepithelioma, esthesioneuroblastoma, ependymoblastoma

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 8.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE1	4
PHASE1/PHASE2	1
PHASE2	1
EARLY_PHASE1	1
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06465199	PHASE1/PHASE2	RECRUITING	Eflornithine (DFMO) and AMXT 1501 for Neuroblastoma, CNS Tumors, and Sarcomas
NCT06861244	PHASE2	RECRUITING	Embryonal Tumor With Multilayered Rosettes
NCT06193759	PHASE1	RECRUITING	Immunotherapy for Malignant Pediatric Brain Tumors Employing Adoptive Cellular Therapy (IMPACT)
NCT07087002	PHASE1	RECRUITING	GPC2-CAR T Cell Therapy for Relapsed or Refractory Medulloblastoma in Children and Young Adults
NCT07390539	PHASE1	NOT_YET_RECRUITING	B7-H3.CD28Z.CART in CNS Neoplasms
NCT03434262	PHASE1	COMPLETED	SJDAWN: St. Jude Children’s Research Hospital Phase 1 Study Evaluating Molecularly-Driven Doublet Therapies for Children and Young Adults With Recurrent Brain Tumors
NCT06942039	EARLY_PHASE1	RECRUITING	Pilot Study of IT Topotecan and Maintenance Chemotherapy for HR-EBTs in Children < 6 Years, Post Consolidation
NCT04794686	Not specified	RECRUITING	ETMR One: An International Registry and Research Platform for Children With Embryonal Tumor With Multilayer Rosettes

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
EFLORNITHINE	4	1
ETOPOSIDE PHOSPHATE	4	1
SONIDEGIB	4	1
EFLORNITHINE, (S)-	2	1
CHEMBL4228794	0	2
CHEMBL4446459	0	1