MCID: CNT115
MIFTS: 23

Central Nervous System Embryonal Tumor

Categories: Fetal diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Central Nervous System Embryonal Tumor

MalaCards integrated aliases for Central Nervous System Embryonal Tumor:

Name: Central Nervous System Embryonal Tumor 58
Central Nervous System Primitive Neuroectodermal Tumor 58
Cns Pnet 58

Characteristics:

Orphanet epidemiological data:

58
central nervous system embryonal tumor
Inheritance: Not applicable; Age of onset: Childhood;

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

UMLS via Orphanet 72 C0206093 C0206663
Orphanet 58 ORPHA251870

Summaries for Central Nervous System Embryonal Tumor

MalaCards based summary : Central Nervous System Embryonal Tumor, also known as central nervous system primitive neuroectodermal tumor, is related to adult central nervous system primitive neuroectodermal neoplasm and childhood central nervous system primitive neuroectodermal neoplasm. The drugs Cisplatin and Dexamethasone acetate have been mentioned in the context of this disorder. Affiliated tissues include brain, liver and endothelial.

Related Diseases for Central Nervous System Embryonal Tumor

Graphical network of the top 20 diseases related to Central Nervous System Embryonal Tumor:



Diseases related to Central Nervous System Embryonal Tumor

Symptoms & Phenotypes for Central Nervous System Embryonal Tumor

Drugs & Therapeutics for Central Nervous System Embryonal Tumor

Drugs for Central Nervous System Embryonal Tumor (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 96)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
2
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
3
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
4
Ondansetron Approved Phase 3 99614-02-5 4595
5
Granisetron Approved, Investigational Phase 3 109889-09-0 3510
6 Gastrointestinal Agents Phase 3
7 Autonomic Agents Phase 3
8 BB 1101 Phase 3
9 Emetics Phase 3
10 Antiemetics Phase 3
11
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
12
Irinotecan Approved, Investigational Phase 2 97682-44-5, 100286-90-6 60838
13
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
14
Etoposide Approved Phase 1, Phase 2 33419-42-0 36462
15
Mechlorethamine Approved, Investigational Phase 1, Phase 2 51-75-2 4033
16
Ifosfamide Approved Phase 1, Phase 2 3778-73-2 3690
17
Carboplatin Approved Phase 1, Phase 2 41575-94-4 10339178 498142 38904
18
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
19
Bevacizumab Approved, Investigational Phase 2 216974-75-3
20
Decitabine Approved, Investigational Phase 1, Phase 2 2353-33-5 451668
21
MK-1775 Investigational Phase 1, Phase 2 955365-80-7, 501-36-0 24856436 445154
22
Camptothecin Experimental Phase 2 7689-03-4
23 topoisomerase I inhibitors Phase 2
24 Topoisomerase Inhibitors Phase 1, Phase 2
25 Alkylating Agents Phase 1, Phase 2
26 Etoposide phosphate Phase 1, Phase 2
27
Isophosphamide mustard Phase 1, Phase 2 0
28 Protein Kinase Inhibitors Phase 1, Phase 2
29 Liver Extracts Phase 1, Phase 2
30 Antibodies, Monoclonal Phase 2
31 Antineoplastic Agents, Immunological Phase 2
32 Endothelial Growth Factors Phase 2
33 Angiogenesis Inhibitors Phase 2
34 Mitogens Phase 2
35 Immunoglobulin G Phase 2
36 Immunoglobulins Phase 2
37 Antibodies Phase 2
38 Immunologic Factors Phase 1, Phase 2
39 Vaccines Phase 1, Phase 2
40 interferons Phase 1, Phase 2
41 Poly ICLC Phase 1, Phase 2
42 Interferon Inducers Phase 1, Phase 2
43 Antimetabolites Phase 1, Phase 2
44
Sargramostim Approved, Investigational Phase 1 123774-72-1, 83869-56-1
45
Vincristine Approved, Investigational Phase 1 2068-78-2, 57-22-7 5978
46
Nicotinamide Approved, Investigational Phase 1 98-92-0 936
47
Sirolimus Approved, Investigational Phase 1 53123-88-9 5284616 6436030 46835353
48
Everolimus Approved Phase 1 159351-69-6 6442177 70789204
49
Miconazole Approved, Investigational, Vet_approved Phase 1 22916-47-8 4189
50
Lenograstim Approved, Investigational Phase 1 135968-09-1

Interventional clinical trials:

(show all 18)
# Name Status NCT ID Phase Drugs
1 SIOP Intracranial Germ Cell Tumours Protocol Completed NCT00293358 Phase 3 carboplatin;cisplatin;etoposide phosphate;ifosfamide
2 Randomized Controlled Trial of Acupressure to Control Chemotherapy-Induced Nausea (CIN) in Children Receiving Highly Emetogenic Chemotherapy Completed NCT01346267 Phase 3
3 Phase II Treatment of Children With Newly Diagnosed Malignant Central Nervous System Tumors With Temozolomide Prior to Radiation Therapy Completed NCT00005955 Phase 2 temozolomide
4 Phase II Pre-Irradiation Chemotherapy for Central Nervous System Germ Cell Malignancies Completed NCT00002472 Phase 2 cisplatin;etoposide
5 A Phase II Study To Assess The Ability Of Neoadjuvant Chemotherapy Plus/Minus Second Look Surgery To Eliminate All Measurable Disease Prior To Radiotherapy For NGGCT Completed NCT00047320 Phase 2 carboplatin;etoposide;ifosfamide;thiotepa
6 A Phase 1/2 Study of AZD1775 (MK-1775) in Combination With Oral Irinotecan in Children, Adolescents, and Young Adults With Relapsed or Refractory Solid Tumors Recruiting NCT02095132 Phase 1, Phase 2 Adavosertib;Irinotecan;Irinotecan Hydrochloride
7 Dasatinib With Ifosfamide, Carboplatin, Etoposide: A Pediatric Phase I/II Trial Active, not recruiting NCT00788125 Phase 1, Phase 2 carboplatin;dasatinib;etoposide phosphate;ifosfamide
8 Temozolomide With Irinotecan Versus Temozolomide, Irinotecan Plus Bevacizumab (NSC# 704865) for Recurrent/Refractory Medulloblastoma/CNS PNET of Childhood, a COG Randomized Phase II Screening Trial Active, not recruiting NCT01217437 Phase 2 Irinotecan Hydrochloride;Temozolomide
9 A Phase I/Pilot II Trial Combining Decitabine and Vaccine Therapy for Patients With Relapsed or Refractory Pediatric High Grade Gliomas, Medulloblastomas, and Central Nervous System Primitive Neuroectodermal Tumors (CNS PNETs) Terminated NCT02332889 Phase 1, Phase 2 Decitabine and Hiltonol
10 Phase I and Pharmacokinetic Trial of PTC299 in Pediatric Patients With Refractory or Recurrent CNS Tumors Completed NCT01158300 Phase 1 VEGF inhibitor PTC299
11 A Pilot Study of Intensive Chemotherapy With Peripheral Stem Cell Support for Infants With Malignant Brain Tumors Completed NCT00003141 Phase 1 carboplatin;cisplatin;cyclophosphamide;etoposide;thiotepa;vincristine sulfate
12 A Phase 1 Study of Imetelstat, a Telomerase Inhibitor, in Children With Refractory or Recurrent Solid Tumors and Lymphomas Completed NCT01273090 Phase 1 imetelstat sodium
13 A Phase I and Surgical Study of Ribociclib and Everolimus (RAD001) in Children With Recurrent or Refractory Malignant Brain Tumors Recruiting NCT03387020 Phase 1 Everolimus;Ribociclib
14 Phase I Dose Escalation Study of N-Acetylcysteine Administered in Conjunction With Carboplatin, Cyclophosphamide, and Etoposide Phosphate BBBD, in Children With Malignant Brain Tumors Terminated NCT00238173 Phase 1 acetylcysteine;carboplatin;cyclophosphamide;etoposide phosphate;mannitol;sodium thiosulfate
15 Follow-up Survey of Patients Who Were Treated for Medulloblastoma or Primitive Neuroectodermal Tumors of the Central Nervous in Norway Completed NCT02851355
16 Multicenter Register for Children and Young Adults With Intracranial Localized Medulloblastoma, Central Nervous System (CNS)- Primitive Neuroectodermal Tumour (PNET), or Ependymoma Completed NCT02238899
17 International HIT-MED Registry (I-HIT-MED) for Children, Adolescents, and Adults With Medulloblastoma, Ependymoma, Pineoblastoma, CNS-primitive Neuroectodermal Tumours Recruiting NCT02417324
18 Evaluation of Cerebral Spinal Fluid (CSF) Proteome and Angiogenesis Proteins in Children With Newly Diagnosed Central Nervous System Tumors Withdrawn NCT00897858

Search NIH Clinical Center for Central Nervous System Embryonal Tumor

Genetic Tests for Central Nervous System Embryonal Tumor

Anatomical Context for Central Nervous System Embryonal Tumor

MalaCards organs/tissues related to Central Nervous System Embryonal Tumor:

40
Brain, Liver, Endothelial, Pineal, Spinal Cord, Kidney

Publications for Central Nervous System Embryonal Tumor

Articles related to Central Nervous System Embryonal Tumor:

(show top 50) (show all 65)
# Title Authors PMID Year
1
Unmet rehabilitation needs in 86% of Norwegian paediatric embryonal brain tumour survivors. 61
31977119 2020
2
Children treated for medulloblastoma and supratentorial primitive neuroectodermal tumor in Norway from 1974 through 2013: Unexplainable regional differences in survival. 61
31264356 2019
3
The oncolytic adenovirus VCN-01 promotes anti-tumor effect in primitive neuroectodermal tumor models. 61
31591461 2019
4
Sleeping Beauty Insertional Mutagenesis Reveals Important Genetic Drivers of Central Nervous System Embryonal Tumors. 61
30674530 2019
5
Extensive Molecular and Clinical Heterogeneity in Patients With Histologically Diagnosed CNS-PNET Treated as a Single Entity: A Report From the Children's Oncology Group Randomized ACNS0332 Trial. 61
30332335 2018
6
Sellar Embryonal Tumor: A Case Report and Review of the Literature. 61
30459893 2018
7
Genetically engineered cerebral organoids model brain tumor formation. 61
30038414 2018
8
Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors. 61
29995179 2018
9
Epigenetic regulators Rbbp4 and Hdac1 are overexpressed in a zebrafish model of RB1 embryonal brain tumor, and are required for neural progenitor survival and proliferation. 61
29914980 2018
10
Management and outcome of children and adolescents with non-medulloblastoma CNS embryonal tumors in Spain: room for improvement in standards of care. 61
29248974 2018
11
Characterization of brain tumor initiating cells isolated from an animal model of CNS primitive neuroectodermal tumors. 61
29568390 2018
12
A primitive neuroectodermal tumor in an adult: Case report of a unique location and MRI characteristics. 61
29443778 2018
13
The case for DNA methylation based molecular profiling to improve diagnostic accuracy for central nervous system embryonal tumors (not otherwise specified) in adults. 61
28993028 2018
14
Outcome for children treated for medulloblastoma and supratentorial primitive neuroectodermal tumor (CNS-PNET) - a retrospective analysis spanning 40 years of treatment. 61
28325133 2017
15
Stabilization of HIF-1α and HIF-2α, up-regulation of MYCC and accumulation of stabilized p53 constitute hallmarks of CNS-PNET animal model. 61
28249000 2017
16
Malignant glioma with primitive neuroectodermal tumor-like component (MG-PNET): novel microarray findings in a pediatric patient. 61
27781423 2016
17
MEK Inhibitors Reverse Growth of Embryonal Brain Tumors Derived from Oligoneural Precursor Cells. 61
27783941 2016
18
Incidence and survival of children and young people with central nervous system embryonal tumours in the North of England, 1990-2013. 61
27151553 2016
19
Histologically distinct neuroepithelial tumors with histone 3 G34 mutation are molecularly similar and comprise a single nosologic entity. 61
26482474 2016
20
Systematic comparison of MRI findings in pediatric ependymoblastoma with ependymoma and CNS primitive neuroectodermal tumor not otherwise specified. 61
25916887 2015
21
Primitive neuroectodermal tumors of the brainstem in children treated according to the HIT trials: clinical findings of a rare disease. 61
25555122 2015
22
A phase I trial of AT9283 (a selective inhibitor of aurora kinases) in children and adolescents with solid tumors: a Cancer Research UK study. 61
25370467 2015
23
Efficacy of cabazitaxel in mouse models of pediatric brain tumors. 61
25140037 2015
24
Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor. 61
25115737 2014
25
Primary intracranial peripheral PNET"--a case report and review. 61
25591683 2014
26
Treatment of children with central nervous system primitive neuroectodermal tumors/pinealoblastomas in the prospective multicentric trial HIT 2000 using hyperfractionated radiation therapy followed by maintenance chemotherapy. 61
24969797 2014
27
[CNS primitive neuroectodermal tumor suspected as a secondary recurrence after radiation therapy for medulloblastoma:a case report]. 61
25006105 2014
28
Intracranial ewings sarcoma/peripheral primitive neuroectodermal tumor. 61
25237951 2014
29
Adults with CNS primitive neuroectodermal tumors/pineoblastomas: results of multimodal treatment according to the pediatric HIT 2000 protocol. 61
24407732 2014
30
A novel C19MC amplified cell line links Lin28/let-7 to mTOR signaling in embryonal tumor with multilayered rosettes. 61
24311633 2014
31
Epigenetic repression of the dopamine receptor D4 in pediatric tumors of the central nervous system. 61
24264533 2014
32
RHPS4 G-quadruplex ligand induces anti-proliferative effects in brain tumor cells. 61
24454961 2014
33
Evolving of therapeutic strategies for CNS-PNET. 61
23852767 2013
34
Treatment Options for Medulloblastoma and CNS Primitive Neuroectodermal Tumor (PNET). 61
23979905 2013
35
Somatostatin receptor subtype 2 (sst₂) is a potential prognostic marker and a therapeutic target in medulloblastoma. 61
23677175 2013
36
The role of the WNT/β-catenin pathway in central nervous system primitive neuroectodermal tumours (CNS PNETs). 61
23591193 2013
37
What's in a name? Intracranial peripheral primitive neuroectodermal tumors and CNS primitive neuroectodermal tumors are not the same. 61
23519360 2013
38
A very rare cancer in Down syndrome: medulloblastoma. Epidemiological data from 13 countries. 61
23307327 2013
39
H3.3 G34R mutations in pediatric primitive neuroectodermal tumors of central nervous system (CNS-PNET) and pediatric glioblastomas: possible diagnostic and therapeutic implications? 61
23354654 2013
40
Treatment of young children with CNS-primitive neuroectodermal tumors/pineoblastomas in the prospective multicenter trial HIT 2000 using different chemotherapy regimens and radiotherapy. 61
23223339 2013
41
Loss of INI1 protein expression defines a subgroup of aggressive central nervous system primitive neuroectodermal tumors. 61
22672440 2013
42
New gene signatures for pediatric brain tumors: a step forward in the understanding of molecular basis of CNS PNET. 61
26835277 2013
43
Malignant peripheral nerve sheath tumor with primitive neuroepithelial differentiation in an adult: a case report. 61
22200660 2012
44
DNA copy number alterations in central primitive neuroectodermal tumors and tumors of the pineal region: an international individual patient data meta-analysis. 61
22772606 2012
45
Antiangiogenic metronomic therapy for children with recurrent embryonal brain tumors. 61
22147459 2012
46
CNS PNET molecular subgroups with distinct clinical features. 61
22691719 2012
47
Markers of survival and metastatic potential in childhood CNS primitive neuro-ectodermal brain tumours: an integrative genomic analysis. 61
22691720 2012
48
Review: In vivo models for defining molecular subtypes of the primitive neuroectodermal tumor genome: current challenges and solutions. 61
22773561 2012
49
Primary spinal extradural Ewing's sarcoma (primitive neuroectodermal tumor): Report of a case and meta-analysis of the reported cases in the literature. 61
22629492 2012
50
Genome-wide molecular characterization of central nervous system primitive neuroectodermal tumor and pineoblastoma. 61
21798848 2011

Variations for Central Nervous System Embryonal Tumor

Expression for Central Nervous System Embryonal Tumor

Search GEO for disease gene expression data for Central Nervous System Embryonal Tumor.

Pathways for Central Nervous System Embryonal Tumor

GO Terms for Central Nervous System Embryonal Tumor

Sources for Central Nervous System Embryonal Tumor

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