Anaplastic pleomorphic xanthoastrocytoma

Summary

Anaplastic pleomorphic xanthoastrocytoma (MONDO:0850312) is a disease and 5 clinical trials. Top therapeutic interventions include trametinib, dabrafenib, and binimetinib. A subtype of astrocytic tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Clinical trials: 5

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	anaplastic pleomorphic xanthoastrocytoma
Mondo ID	MONDO:0850312
DOID	DOID:0080854
NCIT	C129327
UMLS	C4283858
MedGen	924393
GARD	0013624
Is cancer (heuristic)	no

Disease family

This is a subtype of astrocytic 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 › nervous system neoplasm › neuroepithelial neoplasm › glioma › astrocytic tumor › anaplastic pleomorphic xanthoastrocytoma

Related subtypes (7): adult astrocytic tumor, childhood astrocytic tumor, gliofibroma, high grade astrocytic tumor, astrocytoma (excluding glioblastoma), low grade astrocytic tumor, infant-type hemispheric glioma

Subtypes (1): pleomorphic xanthoastrocytoma BRAF mutant

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: 5.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	3
PHASE4	1
PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT03975829	PHASE4	RECRUITING	Pediatric Long-Term Follow-up and Rollover Study
NCT03919071	PHASE2	ACTIVE_NOT_RECRUITING	Dabrafenib Combined With Trametinib After Radiation Therapy in Treating Patients With Newly-Diagnosed High-Grade Glioma
NCT02684058	PHASE2	COMPLETED	Study of Efficacy and Safety of Dabrafenib in Combination With Trametinib in Pediatric Patients With BRAF V600 Mutation Positive LGG or Relapsed or Refractory HGG Tumors
NCT03973918	PHASE2	TERMINATED	Study of Binimetinib With Encorafenib in Adults With Recurrent BRAF V600-Mutated HGG
NCT04541082	PHASE1	RECRUITING	Phase I Study of Oral ONC206 in Recurrent and Rare Primary Central Nervous System Neoplasms

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
TRAMETINIB	4	4
DABRAFENIB	4	3
BINIMETINIB	4	1
ENCORAFENIB	4	1
ONC-206	1	1
CHEMBL5433950	0	2