MCID: SPR008
MIFTS: 42

Supratentorial Primitive Neuroectodermal Tumor

Categories: Cancer diseases, Fetal diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Supratentorial Primitive Neuroectodermal Tumor

MalaCards integrated aliases for Supratentorial Primitive Neuroectodermal Tumor:

Name: Supratentorial Primitive Neuroectodermal Tumor 12 20 6 15 70
Supratentorial Embryonal Tumor, Not Otherwise Specified 20
Ewings Sarcoma-Primitive Neuroectodermal Tumor 70
Supratentorial Pnet 12

Classifications:



External Ids:

Disease Ontology 12 DOID:4791
NCIt 50 C6968
SNOMED-CT 67 699318007
UMLS 70 C0684337 C1336538

Summaries for Supratentorial Primitive Neuroectodermal Tumor

MalaCards based summary : Supratentorial Primitive Neuroectodermal Tumor, also known as supratentorial embryonal tumor, not otherwise specified, is related to cerebral neuroblastoma and pineoblastoma, and has symptoms including fever An important gene associated with Supratentorial Primitive Neuroectodermal Tumor is DICER1 (Dicer 1, Ribonuclease III), and among its related pathways/superpathways are MicroRNAs in cancer and Neuroscience. The drugs Etoposide and Cisplatin have been mentioned in the context of this disorder. Affiliated tissues include brain, pineal and neutrophil, and related phenotypes are behavior/neurological and cellular

Related Diseases for Supratentorial Primitive Neuroectodermal Tumor

Diseases related to Supratentorial Primitive Neuroectodermal Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 257)
# Related Disease Score Top Affiliating Genes
1 cerebral neuroblastoma 30.7 SYP GFAP
2 pineoblastoma 30.5 SYP GFAP DICER1
3 medulloepithelioma 30.2 SYP SMARCB1 GFAP DICER1
4 olfactory neuroblastoma 30.0 SYP SHH S100B CD99
5 ependymoblastoma 29.8 SYP SMARCB1 MYCN GFAP
6 neuroblastoma 29.1 TH SYP SHH RASSF1 NCAM1 MYCN
7 malignant astrocytoma 29.0 S100B OLIG2 MYC IDH1 GFAP
8 pilocytic astrocytoma 28.8 SYP S100B OLIG2 IDH1 GFAP APC
9 medulloblastoma 28.5 SYP SMARCB1 SHH S100B RASSF1 NCAM1
10 brain cancer 27.8 SYP SMARCB1 SHH OLIG2 MYCN MYC
11 childhood supratentorial embryonal tumor, not otherwise specified 11.2
12 myocardium cancer 10.4 SYP CD99
13 central nervous system embryonal tumor 10.4
14 anal neuroendocrine tumor 10.4 SYP NCAM1
15 extraosseous ewings sarcoma-primitive neuroepithelial tumor 10.4 SYP CD99
16 esophageal neuroendocrine tumor 10.4 SYP NCAM1
17 pancreatic serous cystadenocarcinoma 10.4 SYP NCAM1
18 laryngeal small cell carcinoma 10.4 SYP NCAM1
19 gallbladder small cell carcinoma 10.4 SYP NCAM1
20 ureter small cell carcinoma 10.4 SYP NCAM1
21 papillary thymic adenocarcinoma 10.4 SYP NCAM1
22 glomangiosarcoma 10.4 SYP NCAM1
23 urinary bladder small cell neuroendocrine carcinoma 10.4 SYP NCAM1
24 ampulla of vater benign neoplasm 10.4 SYP APC
25 gastric small cell carcinoma 10.4 SYP NCAM1
26 sensory organ benign neoplasm 10.4 SYP NCAM1
27 esophageal adenoid cystic carcinoma 10.4 SYP NCAM1
28 ovarian large-cell neuroendocrine carcinoma 10.4 SYP NCAM1
29 duodenal benign neoplasm 10.4 SYP APC
30 primary hepatic neuroendocrine carcinoma 10.4 SYP S100B
31 cutaneous ganglioneuroma 10.4 SYP NCAM1
32 orbital cancer 10.3 SYP NCAM1
33 small cell osteogenic sarcoma 10.3 SYP CD99
34 biliary tract benign neoplasm 10.3 SYP NCAM1
35 rosette-forming glioneuronal tumor 10.3 SYP S100B
36 prostate stromal sarcoma 10.3 SYP NCAM1
37 endometrial small cell carcinoma 10.3 SYP NCAM1
38 glomangiomatosis 10.3 SYP NCAM1
39 ovarian sex-cord stromal tumor 10.3 DICER1 CD99
40 pulmonary large cell neuroendocrine carcinoma 10.3 SYP NCAM1
41 optic nerve astrocytoma 10.3 SYP SMARCB1
42 nasal cavity olfactory neuroblastoma 10.3 SYP NCAM1
43 askin's tumor 10.3 SYP CD99
44 pineal region teratoma 10.3 SYP SMARCB1
45 breast neuroendocrine neoplasm 10.3 SYP NCAM1
46 small intestine benign neoplasm 10.3 SYP APC
47 intracranial primitive neuroectodermal tumor 10.3 SYP RASSF1 CD99
48 kidney rhabdoid cancer 10.3 SYP SMARCB1
49 extraosseous ewing sarcoma 10.3 SYP NCAM1 CD99
50 retroperitoneal neuroblastoma 10.3 MYCN CD99

Graphical network of the top 20 diseases related to Supratentorial Primitive Neuroectodermal Tumor:



Diseases related to Supratentorial Primitive Neuroectodermal Tumor

Symptoms & Phenotypes for Supratentorial Primitive Neuroectodermal Tumor

UMLS symptoms related to Supratentorial Primitive Neuroectodermal Tumor:


fever

MGI Mouse Phenotypes related to Supratentorial Primitive Neuroectodermal Tumor:

46 (show all 13)
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.34 APC DDC DICER1 GFAP MYC MYCN
2 cellular MP:0005384 10.27 APC DDC DICER1 GFAP MYC MYCN
3 homeostasis/metabolism MP:0005376 10.23 APC DDC DICER1 GFAP IDH1 MYC
4 mortality/aging MP:0010768 10.22 APC DDC DICER1 GFAP IDH1 MYC
5 cardiovascular system MP:0005385 10.21 APC DDC DICER1 GFAP MYC MYCN
6 liver/biliary system MP:0005370 10.03 APC DICER1 MYC MYCN RASSF1 SHH
7 nervous system MP:0003631 10.03 APC DDC DICER1 GFAP MYC MYCN
8 digestive/alimentary MP:0005381 10.02 APC GFAP MYC MYCN RASSF1 SHH
9 muscle MP:0005369 9.97 APC DICER1 GFAP MYC MYCN OLIG2
10 neoplasm MP:0002006 9.92 APC DICER1 MYC OLIG2 RASSF1 SHH
11 normal MP:0002873 9.85 APC DICER1 GFAP MYC MYCN RASSF1
12 skeleton MP:0005390 9.61 APC DDC DICER1 IDH1 MYC MYCN
13 vision/eye MP:0005391 9.32 APC DDC DICER1 GFAP MYCN NCAM1

Drugs & Therapeutics for Supratentorial Primitive Neuroectodermal Tumor

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

(show all 40)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Etoposide Approved Phase 3 33419-42-0 36462
2
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
3
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
4
Lenograstim Approved, Investigational Phase 3 135968-09-1
5
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
6
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
7
Levoleucovorin Approved, Investigational Phase 3 68538-85-2 149436
8
Thiotepa Approved, Investigational Phase 3 52-24-4 5453
9
Carboplatin Approved Phase 3 41575-94-4 10339178 498142 38904
10
leucovorin Approved Phase 3 58-05-9 6006
11
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
12 Etoposide phosphate Phase 3
13 Immunosuppressive Agents Phase 3
14 Alkylating Agents Phase 3
15 Immunologic Factors Phase 3
16 Tubulin Modulators Phase 3
17 Antirheumatic Agents Phase 3
18 Antimitotic Agents Phase 3
19 Vitamin B9 Phase 3
20 Antimetabolites Phase 3
21 Folic Acid Antagonists Phase 3
22 Vitamin B Complex Phase 3
23 Dermatologic Agents Phase 3
24 Folate Phase 3
25 Nutrients Phase 3
26 Micronutrients Phase 3
27 Trace Elements Phase 3
28 Vitamins Phase 3
29 Antidotes Phase 3
30 Protective Agents Phase 3
31 Calcium, Dietary Phase 3
32
Calcium Nutraceutical Phase 3 7440-70-2 271
33
Topotecan Approved, Investigational Phase 2 123948-87-8, 119413-54-6 60700
34
Melphalan Approved Phase 2 148-82-3 4053 460612
35
Oxaliplatin Approved, Investigational Phase 2 61825-94-3 5310940 9887054 43805 6857599
36
Amifostine Approved, Investigational Phase 2 20537-88-6 2141
37 Radiation-Protective Agents Phase 2
38 Fluorides Early Phase 1
39 Fluorodeoxyglucose F18 Early Phase 1
40 Radiopharmaceuticals Early Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Treatment of Patients With Newly Diagnosed Medulloblastoma, Supratentorial Primitive Neuroectodermal Tumor, or Atypical Teratoid Rhabdoid Tumor Active, not recruiting NCT00085202 Phase 3 cisplatin;cyclophosphamide;vincristine
2 A Phase III Randomized Trial for the Treatment of Newly Diagnosed Supratentorial PNET and High Risk Medulloblastoma in Children <36 Months Old With Intensive Induction Chemotherapy With Methotrexate Followed by Consolidation With Stem Cell Rescue Versus the Same Therapy Without Methotrexate Active, not recruiting NCT00336024 Phase 3 etoposide;cyclophosphamide;cisplatin;carboplatin;thiotepa;methotrexate;leucovorin calcium;vincristine sulfate
3 A Phase II Study of Oxaliplatin in Children With Recurrent or Refractory Medulloblastoma, Supratentorial Primitive Neuroectodermal Tumors and Atypical Teratoid Rhabdoid Tumors Completed NCT00047177 Phase 2 Oxaliplatin
4 Dose Intensive Chemotherapy for Patients Greater Than or Equal To 10 Years of Age With Newly Diagnosed High Stage Medulloblastoma, Supratentorial Primitive Neuroectodermal Tumors (PNET) and Ependymoma: A Feasibility Study of an Intensive Induction Chemotherapy Regimen Followed by Standard Irradiation Completed NCT00006258 Phase 2 cisplatin;cyclophosphamide;etoposide;methotrexate;vincristine sulfate
5 Phase II Prospective Study of Sequential Myeloablative Chemotherapy With Stem Cell Rescue for the Treatment of Selected High Risk CNS Tumors and Recurrent CNS Tumors Completed NCT00179803 Phase 2
6 Treatment of Newly Diagnosed Medulloblastoma and Supratentorial PNET in Patients At Least 3 Years With a Phase II Topotecan Window (High-Risk Patients Only), Risk-Adapted Radiation Therapy, and Dose-Intensive Chemotherapy With Peripheral Blood Stem Cell Support Completed NCT00003211 Phase 2 amifostine trihydrate;cisplatin;cyclophosphamide;vincristine sulfate
7 Feasibility of Using Concurrent Carboplatin and Reduced Dose Craniospinal Radiation (24Gy) for Metastatic Medulloblastoma, High-Risk Supratentorial PNET and Metastatic PNET Completed NCT01542736 Phase 2 Carboplatin;Vincristine
8 A Pilot Study of the Utility of 18F-FLT-PET and Diffusion-Weighted MRI for Surgical Planning, Radiotherapy Target Delineation, and Treatment Response Evaluation in Ewing Sarcoma Patients Completed NCT01825902 Early Phase 1 fluorine F 18 fluorothymidine
9 A Pilot Study of Whole-body MRI-guided Intensity Modulated Radiation Therapy Combined With Systemic Chemotherapy Followed by High-Dose Chemotherapy With Busulfan, Melphalan and Topotecan and Stem Cell Rescue in Patients With Poor Risk Ewing's Sarcoma Withdrawn NCT01795430 etoposide;ifosfamide;topotecan hydrochloride;busulfan;melphalan

Search NIH Clinical Center for Supratentorial Primitive Neuroectodermal Tumor

Genetic Tests for Supratentorial Primitive Neuroectodermal Tumor

Anatomical Context for Supratentorial Primitive Neuroectodermal Tumor

MalaCards organs/tissues related to Supratentorial Primitive Neuroectodermal Tumor:

40
Brain, Pineal, Neutrophil, Bone Marrow, Cerebellum, Myeloid

Publications for Supratentorial Primitive Neuroectodermal Tumor

Articles related to Supratentorial Primitive Neuroectodermal Tumor:

(show top 50) (show all 149)
# Title Authors PMID Year
1
Long-Term Control after Radiosurgery for a Recurrent Supratentorial Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature. 61
33326988 2020
2
Neuropsychological functioning in survivors of childhood medulloblastoma/CNS-PNET: The role of secondary medical complications. 61
32729777 2020
3
Pineoblastoma segregates into molecular sub-groups with distinct clinico-pathologic features: a Rare Brain Tumor Consortium registry study. 61
31820118 2020
4
Children treated for medulloblastoma and supratentorial primitive neuroectodermal tumor in Norway from 1974 through 2013: Unexplainable regional differences in survival. 61
31264356 2019
5
Atypical presentation of a germline APC mutation in a child with supratentorial primitive neuroectodermal tumor. 61
30511453 2019
6
Clinical Applications of Quantitative 3-Dimensional MRI Analysis for Pediatric Embryonal Brain Tumors. 61
30108003 2018
7
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
8
Pre-operative neutrophil count and neutrophil-lymphocyte count ratio (NLCR) in predicting the histological grade of paediatric brain tumours: a preliminary study. 61
29188366 2018
9
An unusual biconvex epidural lesion: acutely presenting extraosseous intracranial Ewing's sarcoma. 61
32922892 2018
10
Integrated bioinformatic analysis unveils significant genes and pathways in the pathogenesis of supratentorial primitive neuroectodermal tumor. 61
29670360 2018
11
Outcome for children treated for medulloblastoma and supratentorial primitive neuroectodermal tumor (CNS-PNET) - a retrospective analysis spanning 40 years of treatment. 61
28325133 2017
12
Survival after chemotherapy and stem cell transplant followed by delayed craniospinal irradiation is comparable to upfront craniospinal irradiation in pediatric embryonal brain tumor patients. 61
27778212 2017
13
Suprasellar Primitive Neuroectodermal Tumor in an Adult. 61
26517622 2016
14
Rare Presentation of Supratentorial Primitive Neuroectodermal Tumors Mimicking Bifocal Germ Cell Tumors: 2 Case Reports. 61
26241725 2016
15
Treatment outcome and patterns of failure in patients of non-pineal supratentorial primitive neuroectodermal tumor: review of literature and clinical experience form a regional cancer center in north India. 61
25990846 2015
16
Radiation-Induced Supratentorial Primitive Neuroectodermal Tumor after Treatment for Non-Hodgkin's Lymphoma. 61
26242031 2015
17
The role of stem cells in pediatric central nervous system malignancies. 61
25895707 2015
18
Treatment outcomes and late toxicities in patients with embryonal central nervous system tumors. 61
25209395 2014
19
Tandem high-dose chemotherapy and autologous stem cell rescue in children with newly diagnosed high-risk medulloblastoma or supratentorial primitive neuro-ectodermic tumors. 61
24664937 2014
20
A prognostic analysis of pediatrics central nervous system small cell tumors: evaluation of EGFR family gene amplification and overexpression. 61
24986561 2014
21
A patient tumor-derived orthotopic xenograft mouse model replicating the group 3 supratentorial primitive neuroectodermal tumor in children. 61
24470556 2014
22
Adult supratentorial extra-pineal primitive neuro-ectodermal tumors. 61
24461541 2014
23
Supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in adult. 61
24966562 2014
24
Tropism of the in situ growth from biopsies of childhood neuroectodermal tumors following transplantation into experimental teratoma. 61
24122295 2014
25
Benefit from prolonged dose-intensive chemotherapy for infants with malignant brain tumors is restricted to patients with ependymoma: a report of the Pediatric Oncology Group randomized controlled trial 9233/34. 61
24335695 2014
26
Post-chemotherapy maturation in supratentorial primitive neuroectodermal tumors. 61
24033491 2014
27
Surveillance imaging in children with malignant CNS tumors: low yield of spine MRI. 61
24401959 2014
28
Relapse patterns in pediatric embryonal central nervous system tumors. 61
23921420 2013
29
Proton radiation therapy for pediatric medulloblastoma and supratentorial primitive neuroectodermal tumors: outcomes for very young children treated with upfront chemotherapy. 61
23790826 2013
30
Adult primitive neuroectodermal tumors: the prognostic value of supratentorial location. 61
23720066 2013
31
A supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in a 42-year-old man: a case report and review of the literature. 61
23537064 2013
32
Phase 2 trial of pemetrexed in children and adolescents with refractory solid tumors: a Children's Oncology Group study. 61
22745043 2013
33
Dumbbell-shaped primitive neuroectodermal tumor mimicking trigeminal schwannoma: a case report and review of literature. 61
23546917 2013
34
Supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature. 61
23095172 2012
35
Pilot study of systemic and intrathecal mafosfamide followed by conformal radiation for infants with intracranial central nervous system tumors: a pediatric brain tumor consortium study (PBTC-001). 61
22790443 2012
36
Multiple CDK/CYCLIND genes are amplified in medulloblastoma and supratentorial primitive neuroectodermal brain tumor. 61
22682621 2012
37
Is there a role for high dose chemotherapy with hematopoietic stem cell rescue in patients with relapsed supratentorial PNET? 61
21850536 2012
38
[A supratentorial primitive neuroectodermal tumor exhibiting pathological characteristics of medulloepithelioma: a case report]. 61
22281469 2012
39
Supratentorial primitive neuroectodermal tumor (PNET): an uncommon location. 61
22297899 2011
40
Treatment of a supratentorial primitive neuroectodermal tumor using magnetic resonance-guided laser-induced thermal therapy. 61
22044371 2011
41
Severe allergic reactions to thiol-based cytoprotective agents mesna and amifostine in a child with a supratentorial primitive neuroectodermal tumor. 61
21646919 2011
42
Prognostic significance of cyclin A and B1 in pediatric embryonal tumors. 61
21069428 2011
43
Supratentorial PNET in a young child. 61
21213074 2011
44
Supratentorial primitive neuroectodermal tumors of the central nervous system in adults: molecular and histopathologic analysis of 12 cases. 61
21378543 2011
45
Late complete remission of supratentorial primitive neuroectodermal tumor (CNS-PNET) after multiple relapses. 61
21113945 2011
46
Correlation of MR relative cerebral blood volume measurements with cellular density and proliferation in high-grade gliomas: an image-guided biopsy study. 61
21163880 2011
47
Differential forms of p53 in medulloblastoma primary tumors, cell lines and xenografts. 61
21184030 2011
48
Supratentorial primitive neuroectodermal tumor metastasis to the abdomen via a ventriculoperitoneal shunt. 61
20526591 2010
49
Primitive neuroectodermal tumors in patients with testicular germ cell tumors usually resemble pediatric-type central nervous system embryonal neoplasms and lack chromosome 22 rearrangements. 61
20348883 2010
50
Upregulation of SOX2, NOTCH1, and ID1 in supratentorial primitive neuroectodermal tumors: a distinct differentiation pattern from that of medulloblastomas. 61
20515335 2010

Variations for Supratentorial Primitive Neuroectodermal Tumor

ClinVar genetic disease variations for Supratentorial Primitive Neuroectodermal Tumor:

6
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 DICER1 NM_177438.2(DICER1):c.5425G>A (p.Gly1809Arg) SNV Likely pathogenic 661301 rs1595314951 GRCh37: 14:95557642-95557642
GRCh38: 14:95091305-95091305

Expression for Supratentorial Primitive Neuroectodermal Tumor

Search GEO for disease gene expression data for Supratentorial Primitive Neuroectodermal Tumor.

Pathways for Supratentorial Primitive Neuroectodermal Tumor

Pathways related to Supratentorial Primitive Neuroectodermal Tumor according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 12.25 RASSF1 MYC DICER1 APC
2 12.03 TH SYP S100B NCAM1 MYCN GFAP
3 11.59 OLIG2 MYC GFAP
4 11.46 TH IDH1 DDC
5 11.06 TH SYP SHH S100B OLIG2 NCAM1
6 10.81 TH SHH DDC
7 10.58 TH DDC
8 10.45 TH DDC
9 9.96 MYCN MYC

GO Terms for Supratentorial Primitive Neuroectodermal Tumor

Cellular components related to Supratentorial Primitive Neuroectodermal Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 perinuclear region of cytoplasm GO:0048471 9.02 TH SYP S100B DICER1 APC

Biological processes related to Supratentorial Primitive Neuroectodermal Tumor according to GeneCards Suite gene sharing:

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 cellular response to drug GO:0035690 9.54 TH MYC DDC
2 intermediate filament organization GO:0045109 9.52 SHH GFAP
3 response to growth factor GO:0070848 9.51 TH MYC
4 multicellular organism aging GO:0010259 9.49 TH DDC
5 thalamus development GO:0021794 9.48 SHH OLIG2
6 dopamine biosynthetic process GO:0042416 9.46 TH DDC
7 cellular response to alkaloid GO:0071312 9.43 TH DDC
8 Bergmann glial cell differentiation GO:0060020 9.4 SHH GFAP
9 catecholamine biosynthetic process GO:0042423 9.32 TH DDC
10 response to pyrethroid GO:0046684 9.26 TH DDC
11 aminergic neurotransmitter loading into synaptic vesicle GO:0015842 9.16 TH DDC
12 isoquinoline alkaloid metabolic process GO:0033076 8.96 TH DDC
13 phytoalexin metabolic process GO:0052314 8.62 TH DDC

Molecular functions related to Supratentorial Primitive Neuroectodermal Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.89 TH SYP SMARCB1 SHH S100B RASSF1
2 identical protein binding GO:0042802 9.23 TH SYP S100B RASSF1 OLIG2 NCAM1

Sources for Supratentorial Primitive Neuroectodermal Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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