MCID: CNT112
MIFTS: 24

Central Nervous System Primitive Neuroectodermal Tumor

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Central Nervous System Primitive Neuroectodermal Tumor

MalaCards integrated aliases for Central Nervous System Primitive Neuroectodermal Tumor:

Name: Central Nervous System Primitive Neuroectodermal Tumor 59
Central Nervous System Pnet 59
Cns Pnet 59

Characteristics:

Orphanet epidemiological data:

59
central nervous system primitive neuroectodermal tumor
Inheritance: Not applicable; Age of onset: Childhood;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

UMLS via Orphanet 73 C0206093 C0206663
Orphanet 59 ORPHA251870

Summaries for Central Nervous System Primitive Neuroectodermal Tumor

MalaCards based summary : Central Nervous System Primitive Neuroectodermal Tumor, also known as central nervous system pnet, is related to adult central nervous system primitive neuroectodermal neoplasm and childhood central nervous system primitive neuroectodermal neoplasm. An important gene associated with Central Nervous System Primitive Neuroectodermal Tumor is CADPS (Calcium Dependent Secretion Activator). The drugs Irinotecan and Temozolomide have been mentioned in the context of this disorder. Affiliated tissues include brain, endothelial and spinal cord.

Related Diseases for Central Nervous System Primitive Neuroectodermal Tumor

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



Diseases related to Central Nervous System Primitive Neuroectodermal Tumor

Symptoms & Phenotypes for Central Nervous System Primitive Neuroectodermal Tumor

Drugs & Therapeutics for Central Nervous System Primitive Neuroectodermal Tumor

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

(show all 27)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Irinotecan Approved, Investigational Phase 2 100286-90-6, 97682-44-5 60838
2
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
3
Bevacizumab Approved, Investigational Phase 2 216974-75-3
4
Decitabine Approved, Investigational Phase 1, Phase 2 2353-33-5 451668
5
MK-1775 Investigational Phase 1, Phase 2 501-36-0, 955365-80-7 24856436 445154
6
Camptothecin Experimental Phase 2 7689-03-4
7 Antineoplastic Agents, Phytogenic Phase 2
8 topoisomerase I inhibitors Phase 2
9 Topoisomerase Inhibitors Phase 2
10 Angiogenesis Inhibitors Phase 2
11 Immunoglobulin G Phase 2
12 Alkylating Agents Phase 2
13 Mitogens Phase 2
14 Angiogenesis Modulating Agents Phase 2
15 Antibodies Phase 2
16 Antineoplastic Agents, Immunological Phase 2
17 Immunoglobulins Phase 2
18 Endothelial Growth Factors Phase 2
19 Antibodies, Monoclonal Phase 2
20 Antineoplastic Agents, Alkylating Phase 2
21 Immunologic Factors Phase 1, Phase 2
22 Vaccines Phase 1, Phase 2
23 interferons Phase 1, Phase 2
24 Poly ICLC Phase 1, Phase 2
25 Antimetabolites, Antineoplastic Phase 1, Phase 2
26 Interferon Inducers Phase 1, Phase 2
27 Antimetabolites Phase 1, Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 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
2 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
3 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
4 Follow-up Survey of Patients Who Were Treated for Medulloblastoma or Primitive Neuroectodermal Tumors of the Central Nervous in Norway Completed NCT02851355
5 Multicenter Register for Children and Young Adults With Intracranial Localized Medulloblastoma, Central Nervous System (CNS)- Primitive Neuroectodermal Tumour (PNET), or Ependymoma Completed NCT02238899
6 International HIT-MED Registry (I-HIT-MED) for Children, Adolescents, and Adults With Medulloblastoma, Ependymoma, Pineoblastoma, CNS-primitive Neuroectodermal Tumours Recruiting NCT02417324

Search NIH Clinical Center for Central Nervous System Primitive Neuroectodermal Tumor

Genetic Tests for Central Nervous System Primitive Neuroectodermal Tumor

Anatomical Context for Central Nervous System Primitive Neuroectodermal Tumor

MalaCards organs/tissues related to Central Nervous System Primitive Neuroectodermal Tumor:

41
Brain, Endothelial, Spinal Cord, Pineal, Kidney

Publications for Central Nervous System Primitive Neuroectodermal Tumor

Articles related to Central Nervous System Primitive Neuroectodermal Tumor:

(show top 50) (show all 77)
# Title Authors PMID Year
1
Children treated for medulloblastoma and supratentorial primitive neuroectodermal tumor in Norway from 1974 through 2013: Unexplainable regional differences in survival. 38
31264356 2019
2
Sleeping Beauty Insertional Mutagenesis Reveals Important Genetic Drivers of Central Nervous System Embryonal Tumors. 38
30674530 2019
3
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. 38
30332335 2018
4
Sellar Embryonal Tumor: A Case Report and Review of the Literature. 38
30459893 2018
5
Genetically engineered cerebral organoids model brain tumor formation. 38
30038414 2018
6
Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors. 38
29995179 2018
7
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. 38
29914980 2018
8
Management and outcome of children and adolescents with non-medulloblastoma CNS embryonal tumors in Spain: room for improvement in standards of care. 38
29248974 2018
9
Characterization of brain tumor initiating cells isolated from an animal model of CNS primitive neuroectodermal tumors. 38
29568390 2018
10
A primitive neuroectodermal tumor in an adult: Case report of a unique location and MRI characteristics. 38
29443778 2018
11
Estrogen and soy isoflavonoids decrease sensitivity of medulloblastoma and central nervous system primitive neuroectodermal tumor cells to chemotherapeutic cytotoxicity. 38
28877739 2017
12
Outcome for children treated for medulloblastoma and supratentorial primitive neuroectodermal tumor (CNS-PNET) - a retrospective analysis spanning 40 years of treatment. 38
28325133 2017
13
Stabilization of HIF-1α and HIF-2α, up-regulation of MYCC and accumulation of stabilized p53 constitute hallmarks of CNS-PNET animal model. 38
28249000 2017
14
Malignant glioma with primitive neuroectodermal tumor-like component (MG-PNET): novel microarray findings in a pediatric patient. 38
27781423 2016
15
MEK Inhibitors Reverse Growth of Embryonal Brain Tumors Derived from Oligoneural Precursor Cells. 38
27783941 2016
16
In Utero and Early-Life Exposure to Ambient Air Toxics and Childhood Brain Tumors: A Population-Based Case-Control Study in California, USA. 38
26505805 2016
17
Incidence and survival of children and young people with central nervous system embryonal tumours in the North of England, 1990-2013. 38
27151553 2016
18
Histologically distinct neuroepithelial tumors with histone 3 G34 mutation are molecularly similar and comprise a single nosologic entity. 38
26482474 2016
19
Systematic comparison of MRI findings in pediatric ependymoblastoma with ependymoma and CNS primitive neuroectodermal tumor not otherwise specified. 38
25916887 2015
20
Primitive neuroectodermal tumors of the brainstem in children treated according to the HIT trials: clinical findings of a rare disease. 38
25555122 2015
21
Efficacy of cabazitaxel in mouse models of pediatric brain tumors. 38
25140037 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. 38
25370467 2015
23
Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor. 38
25115737 2014
24
Primary intracranial peripheral PNET"--a case report and review. 38
25591683 2014
25
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. 38
24969797 2014
26
Intracranial ewings sarcoma/peripheral primitive neuroectodermal tumor. 38
25237951 2014
27
[CNS primitive neuroectodermal tumor suspected as a secondary recurrence after radiation therapy for medulloblastoma:a case report]. 38
25006105 2014
28
Adults with CNS primitive neuroectodermal tumors/pineoblastomas: results of multimodal treatment according to the pediatric HIT 2000 protocol. 38
24407732 2014
29
RHPS4 G-quadruplex ligand induces anti-proliferative effects in brain tumor cells. 38
24454961 2014
30
Epigenetic repression of the dopamine receptor D4 in pediatric tumors of the central nervous system. 38
24264533 2014
31
A novel C19MC amplified cell line links Lin28/let-7 to mTOR signaling in embryonal tumor with multilayered rosettes. 38
24311633 2014
32
Embryonal tumor with abundant neuropil and true rosettes: a systematic literature review and report of 2 new cases. 38
23334078 2013
33
Evolving of therapeutic strategies for CNS-PNET. 38
23852767 2013
34
Embryonal tumor with abundant neuropil and true rosettes with melanotic (retinal) differentiation. 38
23688420 2013
35
Anaplastic ependymoma with ependymoblastic multilayered rosettes. 38
23791209 2013
36
Irinotecan, vincristine, cisplatin, cyclophosphamide, and etoposide for refractory or relapsed medulloblastoma/PNET in pediatric patients. 38
23748464 2013
37
Treatment Options for Medulloblastoma and CNS Primitive Neuroectodermal Tumor (PNET). 38
23979905 2013
38
Somatostatin receptor subtype 2 (sst₂) is a potential prognostic marker and a therapeutic target in medulloblastoma. 38
23677175 2013
39
Childhood pheochromocytoma in a survivor of central primitive neuroectodermal tumor. 38
23910809 2013
40
What's in a name? Intracranial peripheral primitive neuroectodermal tumors and CNS primitive neuroectodermal tumors are not the same. 38
23519360 2013
41
The role of the WNT/β-catenin pathway in central nervous system primitive neuroectodermal tumours (CNS PNETs). 38
23591193 2013
42
H3.3 G34R mutations in pediatric primitive neuroectodermal tumors of central nervous system (CNS-PNET) and pediatric glioblastomas: possible diagnostic and therapeutic implications? 38
23354654 2013
43
A very rare cancer in Down syndrome: medulloblastoma. Epidemiological data from 13 countries. 38
23307327 2013
44
A supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in a 42-year-old man: a case report and review of the literature. 38
23537064 2013
45
Treatment of young children with CNS-primitive neuroectodermal tumors/pineoblastomas in the prospective multicenter trial HIT 2000 using different chemotherapy regimens and radiotherapy. 38
23223339 2013
46
Intramedullary spinal cord primitive neuroectodermal tumor presenting with hydrocephalus. 38
22532539 2013
47
Loss of INI1 protein expression defines a subgroup of aggressive central nervous system primitive neuroectodermal tumors. 38
22672440 2013
48
New gene signatures for pediatric brain tumors: a step forward in the understanding of molecular basis of CNS PNET. 38
26835277 2013
49
Malignant peripheral nerve sheath tumor with primitive neuroepithelial differentiation in an adult: a case report. 38
22200660 2012
50
DNA copy number alterations in central primitive neuroectodermal tumors and tumors of the pineal region: an international individual patient data meta-analysis. 38
22772606 2012

Variations for Central Nervous System Primitive Neuroectodermal Tumor

Expression for Central Nervous System Primitive Neuroectodermal Tumor

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

Pathways for Central Nervous System Primitive Neuroectodermal Tumor

GO Terms for Central Nervous System Primitive Neuroectodermal Tumor

Sources for Central Nervous System Primitive Neuroectodermal Tumor

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58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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