Giant cell glioblastoma

Summary

Giant cell glioblastoma (MONDO:0016682) is a disease and 22 clinical trials. Top therapeutic interventions include basiliximab, cabozantinib, and imetelstat sodium. A subtype of brain glioblastoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: <1 / 1 000 000 (Europe) [Orphanet-validated]
• Clinical trials: 22

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.02	Europe	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	giant cell glioblastoma
Mondo ID	MONDO:0016682
Orphanet	251579
DOID	DOID:3074
ICD-11	565094124
NCIT	C4325
UMLS	C0334588
MedGen	83169
GARD	0020705
Is cancer (heuristic)	no

Also known as: Monstrocellular sarcoma [obs]

Data availability: 2 cell lines.

Disease family

This is a subtype of brain glioblastoma. 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 by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › nervous system cancer › central nervous system cancer › brain cancer › brain glioma › brain glioblastoma › giant cell glioblastoma

Related subtypes (2): gliosarcoma, brain stem glioblastoma

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: 22.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	9
PHASE1	7
PHASE1/PHASE2	2
EARLY_PHASE1	2
PHASE3	1
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01480479	PHASE3	COMPLETED	Phase III Study of Rindopepimut/GM-CSF in Patients With Newly Diagnosed Glioblastoma
NCT01095094	PHASE2	TERMINATED	Ritonavir and Lopinavir in Treating Patients With Progressive or Recurrent High-Grade Glioma
NCT01149850	PHASE2	COMPLETED	Bevacizumab and Temozolomide in Treating Older Patients With Newly-Diagnosed Glioblastoma Multiforme or Gliosarcoma
NCT01498328	PHASE2	COMPLETED	A Study of Rindopepimut/GM-CSF in Patients With Relapsed EGFRvIII-Positive Glioblastoma
NCT01514201	PHASE1/PHASE2	COMPLETED	Veliparib, Radiation Therapy, and Temozolomide in Treating Younger Patients With Newly Diagnosed Diffuse Pontine Gliomas
NCT01609790	PHASE2	COMPLETED	Bevacizumab With or Without Trebananib in Treating Patients With Recurrent Brain Tumors
NCT01836549	PHASE2	TERMINATED	Imetelstat Sodium in Treating Younger Patients With Recurrent or Refractory Brain Tumors
NCT02366728	PHASE2	COMPLETED	DC Migration Study for Newly-Diagnosed GBM
NCT03014804	PHASE2	WITHDRAWN	Autologous Dendritic Cells Pulsed With Tumor Lysate Antigen Vaccine and Nivolumab in Treating Patients With Recurrent Glioblastoma
NCT03027388	PHASE2	COMPLETED	Protein Phosphatase 2A Inhibitor, in Recurrent Glioblastoma
NCT04482933	PHASE2	WITHDRAWN	HSV G207 With a Single Radiation Dose in Children With Recurrent High-Grade Glioma
NCT05218408	PHASE1/PHASE2	WITHDRAWN	CYNK-001 IV and IC in Combination With IL2 in Surgical Eligible Recurrent GBM With IDH-1 Wild Type
NCT00960492	PHASE1	COMPLETED	Safety Study of XL184 (Cabozantinib) in Combination With Temozolomide and Radiation Therapy in the Initial Treatment of Adults With Glioblastoma
NCT01082926	PHASE1	COMPLETED	Phase I Study of Cellular Immunotherapy for Recurrent/Refractory Malignant Glioma Using Intratumoral Infusions of GRm13Z40-2, An Allogeneic CD8+ Cytolitic T-Cell Line Genetically Modified to Express the IL 13-Zetakine and HyTK and to be Resistant to Glucocorticoids, in Combination With Interleukin-2
NCT01250470	PHASE1	COMPLETED	Vaccine Therapy and Sargramostim in Treating Patients With Malignant Glioma
NCT01975116	PHASE1	COMPLETED	p28 in Treating Younger Patients With Recurrent or Progressive Central Nervous System Tumors
NCT02709226	PHASE1	COMPLETED	Dose Escalation Trial of Re-irradiation in Good Prognosis Recurrent Glioblastoma
NCT04489420	PHASE1	TERMINATED	Natural Killer Cell (CYNK-001) IV Infusion or IT Administration in Adults With Recurrent GBM
NCT06806228	PHASE1	SUSPENDED	Phase I Pilot Study to Evaluate the Anti-glioblastoma Effect of S-Gboxin in Standard Treatment of Glioblastoma/Diffuse Midline Glioma and Response to Treatment (Regardless of Mutation Status)
NCT01957956	EARLY_PHASE1	COMPLETED	Vaccine Therapy and Temozolomide in Treating Patients With Newly Diagnosed Glioblastoma
NCT03360708	EARLY_PHASE1	COMPLETED	Vaccine Therapy in Treating Patients With Recurrent Glioblastoma
NCT00943462	Not specified	WITHDRAWN	Study of 18F-FEC for Positron Emission Tomography-Computed Tomography (PET-CT) Imaging of GBM

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
BASILIXIMAB	4	1
CABOZANTINIB	4	1
IMETELSTAT SODIUM	4	1
TEMOZOLOMIDE	4	1
RINDOPEPIMUT	3	2
VELIPARIB	3	2
TREBANANIB	3	1
LB-100	2	1
CHEMBL4228794	0	7
CHEMBL4248195	0	1
CHEMBL4787699	0	1