Childhood central nervous system germ cell tumor
diseaseOn this page
Also known as central nervous system childhood germ cell tumorcentral nervous system germ cell tumorcentral nervous system germ cell tumourcentral nervous system paediatric germ cell cancercentral nervous system pediatric germ cell cancerchildhood central nervous system germ cell neoplasmchildhood CNS germ cell neoplasmchildhood CNS germ cell tumorchildhood CNS germ cell tumourchildhood germ cell neoplasm of central nervous systemchildhood germ cell neoplasm of CNSchildhood germ cell neoplasm of the central nervous systemchildhood germ cell neoplasm of the CNSchildhood germ cell tumor of central nervous systemchildhood germ cell tumor of CNSchildhood germ cell tumor of the central nervous systemchildhood germ cell tumor of the CNSchildhood germ cell tumour of central nervous systemchildhood germ cell tumour of CNS
Summary
Childhood central nervous system germ cell tumor (MONDO:0003750) is a cancer (an umbrella term covering 8 Mondo subtypes) and 18 clinical trials. Top therapeutic interventions include irinotecan hydrochloride, pazopanib hydrochloride, and romidepsin. A subtype of central nervous system germ cell tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Umbrella term: 8 Mondo subtypes
- Clinical trials: 18
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | childhood central nervous system germ cell tumor |
| Mondo ID | MONDO:0003750 |
| DOID | DOID:6052 |
| NCIT | C6205 |
| UMLS | C0278754 |
| MedGen | 124505 |
| GARD | 0023650 |
| Anatomy (UBERON) | UBERON:0001017 |
| Is cancer (heuristic) | yes |
Also known as: central nervous system childhood germ cell tumor · central nervous system germ cell tumor · central nervous system germ cell tumour · central nervous system paediatric germ cell cancer · central nervous system pediatric germ cell cancer · childhood central nervous system germ cell neoplasm · childhood central nervous system germ cell tumor · childhood CNS germ cell neoplasm · childhood CNS germ cell tumor · childhood CNS germ cell tumour · childhood germ cell neoplasm of central nervous system · childhood germ cell neoplasm of CNS · childhood germ cell neoplasm of the central nervous system · childhood germ cell neoplasm of the CNS · childhood germ cell tumor of central nervous system · childhood germ cell tumor of CNS · childhood germ cell tumor of the central nervous system · childhood germ cell tumor of the CNS · childhood germ cell tumour of central nervous system · childhood germ cell tumour of CNS (+25 more)
Disease family
This is a subtype of central nervous system germ cell 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 body system or component › nervous system disorder › central nervous system disorder › central nervous system neoplasm › central nervous system germ cell tumor › childhood central nervous system germ cell tumor
Related subtypes (5): malignant pineal area germ cell neoplasm, central nervous system germinoma, adult central nervous system germ cell tumor, mixed germ cell tumor of central nervous system, central nervous system nongerminomatous germ cell tumor
Subtypes (8): central nervous system endodermal sinus tumor, childhood central nervous system mature teratoma, pediatric CNS choriocarcinoma, childhood central nervous system immature teratoma, childhood central nervous system embryonal carcinoma, childhood germ cell brain tumor, childhood central nervous system mixed germ cell tumor, childhood central nervous system germinoma
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: 18.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE1 | 14 |
| PHASE2 | 2 |
| Not specified | 2 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT00004078 | PHASE2 | COMPLETED | Irinotecan in Treating Children With Refractory Solid Tumors |
| NCT00091182 | PHASE2 | COMPLETED | Oxaliplatin in Treating Young Patients With Recurrent Solid Tumors That Have Not Responded to Previous Treatment |
| NCT00052780 | PHASE1 | COMPLETED | Temozolomide and O6-Benzylguanine in Treating Children With Recurrent Brain Tumors |
| NCT00053963 | PHASE1 | COMPLETED | FR901228 in Treating Children With Refractory or Recurrent Solid Tumors or Leukemia |
| NCT00063973 | PHASE1 | COMPLETED | Cilengitide in Treating Children With Refractory Primary Brain Tumors |
| NCT00100880 | PHASE1 | COMPLETED | Lenalidomide in Treating Young Patients With Recurrent, Progressive, or Refractory CNS Tumors |
| NCT00101270 | PHASE1 | COMPLETED | Oxaliplatin and Irinotecan in Treating Young Patients With Refractory Solid Tumors or Lymphomas |
| NCT00281944 | PHASE1 | COMPLETED | Combination Chemotherapy in Treating Young Patients With Advanced Solid Tumors |
| NCT00326664 | PHASE1 | COMPLETED | AZD2171 in Treating Young Patients With Recurrent, Progressive, or Refractory Primary CNS Tumors |
| NCT00363272 | PHASE1 | COMPLETED | Ispinesib in Treating Young Patients With Relapsed or Refractory Solid Tumors or Lymphoma |
| NCT00387920 | PHASE1 | COMPLETED | Sunitinib in Treating Young Patients With Refractory Solid Tumors |
| NCT00638898 | PHASE1 | COMPLETED | Busulfan, Melphalan, Topotecan Hydrochloride, and a Stem Cell Transplant in Treating Patients With Newly Diagnosed or Relapsed Solid Tumor |
| NCT00929903 | PHASE1 | COMPLETED | Pazopanib Hydrochloride in Treating Young Patients With Solid Tumors That Have Relapsed or Not Responded to Treatment |
| NCT00946335 | PHASE1 | COMPLETED | ABT-888 and Temozolomide in Treating Young Patients With Recurrent or Refractory CNS Tumors |
| NCT00994500 | PHASE1 | COMPLETED | Vorinostat and Bortezomib in Treating Young Patients With Refractory or Recurrent Solid Tumors, Including Central Nervous System Tumors and Lymphoma |
| NCT04521946 | PHASE1 | WITHDRAWN | Chemotherapy and Donor Stem Transplant for the Treatment of Patients With High Grade Brain Cancer |
| NCT01049230 | Not specified | ACTIVE_NOT_RECRUITING | Proton Beam Radiation Therapy for Central Nervous System (CNS) Germ Cell Tumors |
| NCT00919750 | Not specified | COMPLETED | Collecting and Storing Blood and Brain Tumor Tissue Samples From Children With Brain Tumors |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| IRINOTECAN HYDROCHLORIDE | 4 | 2 |
| PAZOPANIB HYDROCHLORIDE | 4 | 1 |
| ROMIDEPSIN | 4 | 1 |
| VELIPARIB | 3 | 2 |
| 6-O-BENZYLGUANINE | 3 | 1 |
| CEDIRANIB MALEATE | 3 | 1 |
| CILENGITIDE | 3 | 1 |
| ISPINESIB | 2 | 1 |
| CHEMBL541887 | 0 | 1 |
| CHEMBL4066465 | 0 | 1 |
| CHEMBL2326523 | 0 | 1 |
| CHEMBL3109278 | 0 | 1 |