Gliosarcoma

Summary

Gliosarcoma (MONDO:0016681) is a disease and 136 clinical trials. Molecularly, TPR::NTRK1 Fusion confers sensitivity to Larotrectinib in Gliosarcoma (CIViC Level C). Top therapeutic interventions include bevacizumab, temozolomide, and erlotinib. 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: 136
• Precision-medicine evidence (CIViC): 1 subtype–drug association

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.03	Europe	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	gliosarcoma
Mondo ID	MONDO:0016681
EFO	EFO:1001465
MeSH	D018316
Orphanet	251576
DOID	DOID:3071
NCIT	C3796
UMLS	C0206726
MedGen	64638
GARD	0005653
MedDRA	10018340
Is cancer (heuristic)	no

Also known as: glioblastoma with a sarcomatous component · gliosarcoma · sarcomatous glioblastoma

Data availability: 21 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 › gliosarcoma

Related subtypes (2): giant cell glioblastoma, 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

Drugs indicated for this disease

0 approved, 5 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

Drug	Development status
Bevacizumab	Phase 3 (in late-stage trials)
Depatuxizumab Mafodotin	Phase 3 (in late-stage trials)
Lomustine	Phase 3 (in late-stage trials)
Rindopepimut	Phase 3 (in late-stage trials)
Temozolomide	Phase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): 6-O-BENZYLGUANINE, Aflibercept, Bortezomib, Carboplatin, Carboxyamidotriazole, Carmustine, Cilengitide, Dasatinib Anhydrous, Dovitinib, Erlotinib, Etoposide, Fenretinide, Filgrastim, Gefitinib, Hydroxyurea, Incomplete Freund’S Adjuvant, Ipilimumab, Irinotecan, Lenalidomide, Nintedanib, Nivolumab, Oxygen, Pazopanib, Pembrolizumab, Plerixafor, Rilotumumab, Sargramostim, Sirolimus, Sorafenib, Temsirolimus, Tipifarnib, Topotecan, Trebananib, Vandetanib, Veliparib, Vismodegib, Vitespen, Vorinostat.

Clinical trials & evidence

Clinical trials

Clinical trials: 136.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	55
PHASE1	49
PHASE1/PHASE2	15
EARLY_PHASE1	6
Not specified	5
PHASE3	4
PHASE2/PHASE3	2

Top trials by phase / activity

NCT	Phase	Status	Title
NCT02152982	PHASE2/PHASE3	ACTIVE_NOT_RECRUITING	Temozolomide With or Without Veliparib in Treating Patients With Newly Diagnosed Glioblastoma Multiforme
NCT05095376	PHASE3	ACTIVE_NOT_RECRUITING	Testing the Addition of the Chemotherapy Drug Lomustine (Gleostine) to the Usual Treatment (Temozolomide and Radiation Therapy) for Newly Diagnosed MGMT Methylated Glioblastoma
NCT00884741	PHASE3	COMPLETED	Temozolomide and Radiation Therapy With or Without Bevacizumab in Treating Patients With Newly Diagnosed Glioblastoma
NCT01480479	PHASE3	COMPLETED	Phase III Study of Rindopepimut/GM-CSF in Patients With Newly Diagnosed Glioblastoma
NCT02573324	PHASE3	COMPLETED	A Study of ABT-414 in Participants With Newly Diagnosed Glioblastoma (GBM) With Epidermal Growth Factor Receptor (EGFR) Amplification
NCT04396860	PHASE2/PHASE3	COMPLETED	Testing the Use of the Immunotherapy Drugs Ipilimumab and Nivolumab Plus Radiation Therapy Compared to the Usual Treatment (Temozolomide and Radiation Therapy) for Newly Diagnosed MGMT Unmethylated Glioblastoma
NCT01903330	PHASE2	ACTIVE_NOT_RECRUITING	ERC1671/GM-CSF/Cyclophosphamide for the Treatment of Glioblastoma Multiforme
NCT02337686	PHASE2	ACTIVE_NOT_RECRUITING	Pembrolizumab in Treating Patients With Recurrent Glioblastoma
NCT02455557	PHASE2	ACTIVE_NOT_RECRUITING	SurVaxM Vaccine Therapy and Temozolomide in Treating Patients With Newly Diagnosed Glioblastoma
NCT03197506	PHASE2	ACTIVE_NOT_RECRUITING	Pembrolizumab and Standard Therapy in Treating Patients With Glioblastoma
NCT04528680	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Ultrasound-based Blood-brain Barrier Opening and Albumin-bound Paclitaxel and Carboplatin for Recurrent Glioblastoma
NCT05039281	PHASE1/PHASE2	RECRUITING	Atezolizumab and Cabozantinib for the Treatment of Recurrent Glioblastoma
NCT05084430	PHASE1/PHASE2	RECRUITING	Study of Pembrolizumab and M032 (NSC 733972)
NCT05465954	PHASE2	RECRUITING	Efineptakin Alfa and Pembrolizumab for the Treatment of Recurrent Glioblastoma
NCT05864534	PHASE2	RECRUITING	Phase 2a Immune Modulation With Ultrasound for Newly Diagnosed Glioblastoma
NCT07301268	PHASE2	RECRUITING	GI-102 Alone or With Pembrolizumab Before Surgery for Treatment of Recurrent or Progressive IDH Wildtype Glioblastoma and IDH Mutated Grade 4 Astrocytoma
NCT00100802	PHASE2	COMPLETED	Radiation Therapy, Temozolomide, and Lomustine in Treating Young Patients With Newly Diagnosed Gliomas
NCT00107003	PHASE2	COMPLETED	GW572016 to Treat Recurrent Malignant Brain Tumors
NCT00114309	PHASE2	UNKNOWN	131-I-TM-601 Study in Adults With Recurrent High-Grade Glioma
NCT00165477	PHASE2	COMPLETED	Study of Lenalidomide and XRT in Patients With Newly Diagnosed Glioblastoma Multiforme
NCT00187486	PHASE2	COMPLETED	Safety and Efficacy Study of Tarceva, Temodar, and Radiation Therapy in Patients With Newly Diagnosed Brain Tumors
NCT00354913	PHASE2	COMPLETED	Imatinib Mesylate and Hydroxyurea in Treating Patients With Recurrent or Progressive Meningioma
NCT00365222	PHASE2	TERMINATED	Phase II Study of Temozolomide in Newly Diagnosed Glioblastoma
NCT00402116	PHASE1/PHASE2	COMPLETED	Phase 1/2 Study of Enzastaurin in Newly Diagnosed Glioblastoma Multiforme (GBM) and Gliosarcoma (GS) Patients
NCT00441142	PHASE1/PHASE2	COMPLETED	Zactima With Temodar During Radiation Treatment for Newly Diagnosed Stage IV Brain Tumors
NCT00525525	PHASE2	COMPLETED	Study of Bevacizumab Plus Temodar and Tarceva in Patients With Glioblastoma or Gliosarcoma
NCT00597402	PHASE2	COMPLETED	Avastin in Combination With Radiation (XRT) & Temozolomide, Followed by Avastin, Temozolomide and Irinotecan for Glioblastoma (GBM) and Gliosarcomas
NCT00611325	PHASE2	COMPLETED	Phase II Avastin + Bortezomib for Patients With Recurrent Malignant Glioma
NCT00612339	PHASE2	COMPLETED	Avastin in Combination With Temozolomide for Unresectable or Multifocal GBMs and Gliosarcomas
NCT00612430	PHASE2	COMPLETED	Ph II Bevacizumab + Etoposide for Pts w Recurrent MG
NCT00613028	PHASE2	COMPLETED	Ph II Bev + Either Temozolomide/Etoposide for GBM Pts Who Have Failed Bev + Irinotecan
NCT00615927	PHASE2	COMPLETED	Phase II Imatinib + Hydroxyurea in Treatment of Patients With Recurrent/Progressive Grade II Low-Grade Glioma (LGG)
NCT00657267	PHASE2	COMPLETED	Dose-Intense Temozolomide in Recurrent Glioblastoma
NCT00667394	PHASE2	COMPLETED	Tandutinib Plus Bevacizumab to Treat Recurrent Brain Tumors
NCT00669669	PHASE1/PHASE2	TERMINATED	O6-Benzylguanine-Mediated Tumor Sensitization With Chemoprotected Autologous Stem Cell in Treating Patients With Malignant Gliomas
NCT00671970	PHASE2	COMPLETED	Phase (Ph) II Bevacizumab + Erlotinib for Patients (Pts) With Recurrent Malignant Glioma (MG)
NCT00672243	PHASE2	COMPLETED	Ph II Erlotinib + Sirolimus for Pts w Recurrent Malignant Glioma Multiforme
NCT00735436	PHASE2	TERMINATED	A Study of Gliadel Followed by Avastin + Irinotecan for Glioblastoma Multiforme (GBM)
NCT00879437	PHASE2	COMPLETED	Valproic Acid, Radiation, and Bevacizumab in Children With High Grade Gliomas or Diffuse Intrinsic Pontine Glioma
NCT00883298	PHASE2	COMPLETED	Bi-weekly Temozolomide Plus Bevacizumab for Adult Patients With Recurrent Glioblastoma Multiforme

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
BEVACIZUMAB	4	27
TEMOZOLOMIDE	4	6
ERLOTINIB	4	4
PLERIXAFOR	4	4
VANDETANIB	4	4
LAPATINIB	4	3
LOMUSTINE	4	3
HYDROXYUREA	4	2
LONAFARNIB	4	2
BINIMETINIB	4	1
CABOZANTINIB S-MALATE	4	1
CAPMATINIB	4	1
CHLOROQUINE	4	1
ENCORAFENIB	4	1
ETOPOSIDE PHOSPHATE	4	1
IMETELSTAT SODIUM	4	1
IPILIMUMAB	4	1
LIOTHYRONINE	4	1
MEBENDAZOLE	4	1
MEFLOQUINE	4	1
MEMANTINE HYDROCHLORIDE	4	1
METFORMIN HYDROCHLORIDE	4	1
METHIMAZOLE	4	1
NINTEDANIB	4	1
PALBOCICLIB	4	1
PAZOPANIB	4	1
VALPROIC ACID	4	1
VINCRISTINE	4	1
VORINOSTAT	4	1
VELIPARIB	3	4

Precision-medicine subtype map (CIViC)

Drug × molecular subtype: 1 predictive associations from 1 curated evidence items; also 1 oncogenic.

Molecular subtype	Therapy	Effect	Level	CIViC
TPR::NTRK1 Fusion	Larotrectinib	Sensitivity/Response	CIViC C	EID11746