MCID: HGH044
MIFTS: 40

High Grade Ependymoma

Categories: Bone diseases, Cancer diseases, Neuronal diseases

Aliases & Classifications for High Grade Ependymoma

MalaCards integrated aliases for High Grade Ependymoma:

Name: High Grade Ependymoma 12
Malignant Ependymoma 12 15
Ependymal Neoplasm 12 17
Ependymal Tumors 12 54
Experimental Organism Malignant Ependymoma 70
Anaplastic Ependymoma 70
Ependymoma of Brain 70
Ependymal Tumor 70

Classifications:



External Ids:

Disease Ontology 12 DOID:5074
NCIt 50 C6770
UMLS 70 C0238029 C0280788 C1333407 more

Summaries for High Grade Ependymoma

Disease Ontology : 12 A high grade glioma that is derived from ependymal cells, a type of glial cell, located in ventricle lining within the central part of the brain.

MalaCards based summary : High Grade Ependymoma, also known as malignant ependymoma, is related to supratentorial ependymoma and spinal cord ependymoma, and has symptoms including seizures, vomiting and headache. An important gene associated with High Grade Ependymoma is NF2 (Neurofibromin 2), and among its related pathways/superpathways are Signaling by ERBB4 and Spinal Cord Injury. The drugs Cisplatin and Vincristine have been mentioned in the context of this disorder. Affiliated tissues include ventricle lining, brain and spinal cord, and related phenotypes are Decreased p24 protein expression and Increased proliferation

Related Diseases for High Grade Ependymoma

Diseases related to High Grade Ependymoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 134)
# Related Disease Score Top Affiliating Genes
1 supratentorial ependymoma 32.1 ZFTA YAP1 RELA MAMLD1
2 spinal cord ependymoma 31.9 ZFTA YAP1 NF2 NELL2 FAM118B
3 angiocentric glioma 30.5 IDH1 GFAP
4 anaplastic ependymoma 30.5 RELA NF2 GFAP
5 clear cell ependymoma 30.3 ZFTA NF2 IDH1 GFAP
6 myxopapillary ependymoma 30.1 ZFTA YAP1 RELA PLA2G5 NF2 GFAP
7 benign ependymoma 29.8 ZFTA NF2 KIAA1549 IDH1 GFAP
8 cellular ependymoma 29.8 ZFTA RELA NF2 MGMT GFAP EGFR
9 chordoma 29.7 TP53 GFAP EGFR
10 atypical teratoid rhabdoid tumor 29.7 ZFTA TP53 NF2 IDH1 GFAP
11 oligodendroglioma 29.6 TP53 MGMT IDH1 GFAP EGFR
12 mixed glioma 29.4 TP53 MGMT IDH1 GFAP EGFR
13 meningioma, familial 29.4 TP53 NF2 KIAA1549 GFAP EGFR
14 medulloblastoma 29.2 YAP1 TP53 MGMT IDH1 GFAP EGFR
15 malignant astrocytoma 28.9 TP53 RELA MGMT KIAA1549 IDH1 GFAP
16 posterior fossa ependymoma 11.2
17 brain stem ependymoma 11.2
18 glial tumor 11.1
19 papillary ependymoma 10.5 NF2 GFAP
20 rela fusion-positive ependymoma 10.4 ZFTA RELA
21 suprasellar meningioma 10.4 NF2 GFAP
22 extraventricular neurocytoma 10.3 IDH1 GFAP
23 pediatric supratentorial ependymoma 10.3 ZFTA RELA MAMLD1
24 cerebral convexity meningioma 10.3 NF2 IDH1
25 oligoastrocytoma 10.3 MGMT GFAP
26 malignant peritoneal mesothelioma 10.3 NF2 EGFR
27 chordoid meningioma 10.3 NF2 GFAP
28 rhabdoid meningioma 10.3 NF2 GFAP
29 frontal convexity meningioma 10.3 NF2 IDH1 GFAP
30 urethra transitional cell carcinoma 10.2 TP53 GFAP
31 adult brain stem glioma 10.2 MGMT IDH1
32 pediatric meningioma 10.2 NF2 KIAA1549
33 temporal lobe neoplasm 10.2 MGMT IDH1
34 neuroma 10.2 NF2 MGMT GFAP
35 adult malignant schwannoma 10.2 TP53 NF2
36 chordoid glioma 10.2 TP53 NF2 GFAP
37 choroid plexus cancer 10.2 ZFTA TP53 GFAP
38 tanycytic ependymoma 10.2 ZFTA RELA NF2 MAMLD1 GFAP
39 myeloproliferative syndrome, transient 10.2 YAP1 TP53 NF2
40 laryngeal neuroendocrine tumor 10.1 TP53 IDH1
41 esophagus verrucous carcinoma 10.1 TP53 EGFR
42 optic nerve neoplasm 10.1 NF2 KIAA1549
43 esophageal basaloid squamous cell carcinoma 10.1 TP53 EGFR
44 cerebrovascular benign neoplasm 10.1 TP53 GFAP
45 subependymoma 10.1
46 gemistocytic astrocytoma 10.1 TP53 IDH1 GFAP
47 adult oligodendroglioma 10.1 KIAA1549 IDH1
48 pilocytic astrocytoma of cerebellum 10.1 KIAA1549 IDH1
49 soft tissue sarcoma 10.1 TP53 EGFR
50 breast malignant phyllodes tumor 10.1 TP53 EGFR

Graphical network of the top 20 diseases related to High Grade Ependymoma:



Diseases related to High Grade Ependymoma

Symptoms & Phenotypes for High Grade Ependymoma

UMLS symptoms related to High Grade Ependymoma:


seizures; vomiting; headache; nausea

GenomeRNAi Phenotypes related to High Grade Ependymoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased p24 protein expression GR00163-A-1 9.26 EGFR IDH1 NF2 RELA
2 Increased proliferation GR00094-A 8.62 NF2 TP53

MGI Mouse Phenotypes related to High Grade Ependymoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 respiratory system MP:0005388 9.23 CBY1 EGFR IDH1 MGMT NF2 RELA

Drugs & Therapeutics for High Grade Ependymoma

Drugs for High Grade Ependymoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 44)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
2
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
3
Etoposide Approved Phase 3 33419-42-0 36462
4
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
5
Carboplatin Approved Phase 3 41575-94-4 10339178 498142 38904
6 Tubulin Modulators Phase 3
7 Etoposide phosphate Phase 3
8 Immunologic Factors Phase 3
9 Immunosuppressive Agents Phase 3
10 Antirheumatic Agents Phase 3
11 Keratolytic Agents Phase 3
12 Alkylating Agents Phase 3
13 Dermatologic Agents Phase 3
14 Antimitotic Agents Phase 3
15 Podophyllotoxin Phase 3 518-28-5
16 Proteasome Inhibitors Phase 2
17
Fludarabine Approved Phase 1 21679-14-1, 75607-67-9 30751
18
Melphalan Approved Phase 1 148-82-3 4053 460612
19
Tacrolimus Approved, Investigational Phase 1 104987-11-3 6473866 445643 439492
20
Mechlorethamine Approved, Investigational Phase 1 51-75-2 4033
21
Mycophenolic acid Approved Phase 1 24280-93-1 446541
22
Thiotepa Approved, Investigational Phase 1 52-24-4 5453
23
Phenylalanine Approved, Investigational, Nutraceutical Phase 1 63-91-2 6140
24 Antibiotics, Antitubercular Phase 1
25 Antibodies Phase 1
26 Immunoglobulins Phase 1
27 Immunoglobulins, Intravenous Phase 1
28 gamma-Globulins Phase 1
29 Anti-Bacterial Agents Phase 1
30 Cyclosporins Phase 1
31 Thymoglobulin Phase 1
32 Antitubercular Agents Phase 1
33 Antilymphocyte Serum Phase 1
34 Antimetabolites Phase 1
35 Anti-Infective Agents Phase 1
36 Nitrogen Mustard Compounds Phase 1
37 Rho(D) Immune Globulin Phase 1
38 Calcineurin Inhibitors Phase 1
39
L-Alanine Nutraceutical Phase 1 56-41-7 5950
40
Dopamine Approved 62-31-7, 51-61-6 681
41 Dopamine Agents
42 Neurotransmitter Agents
43 Fluorides
44 Dihydroxyphenylalanine

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase III Randomized Trial of Post-Radiation Chemotherapy in Patients With Newly Diagnosed Ependymoma Ages 1 to 21 Years Active, not recruiting NCT01096368 Phase 3 Carboplatin;Cisplatin;Cyclophosphamide;Etoposide;Vincristine
2 Phase II Clinical Trial of Marizomib for Recurrent Low-Grade and Anaplastic Supratentorial, Infratentorial and Spinal Cord Ependymoma Recruiting NCT03727841 Phase 2 Marizomib
3 Pilot Trial of LET Optimized IMPT for Pediatric Patients With Ependymoma Recruiting NCT03750513 Phase 1
4 A Pilot Study of Allogeneic Hematopoietic Cell Transplantation for Patients With High Grade Central Nervous System Malignancies Recruiting NCT04521946 Phase 1 Etoposide;Fludarabine Phosphate;Melphalan;Mycophenolate Mofetil;Tacrolimus;Thiotepa
5 18F-FDOPA PET/CT or PET/MRI in Patients With Gliomas Terminated NCT02175745 18F-fluoro-dihydroxyphenylalanine

Search NIH Clinical Center for High Grade Ependymoma

Genetic Tests for High Grade Ependymoma

Anatomical Context for High Grade Ependymoma

The Foundational Model of Anatomy Ontology organs/tissues related to High Grade Ependymoma:

19
Ventricle Lining

MalaCards organs/tissues related to High Grade Ependymoma:

40
Brain, Spinal Cord, Endothelial, Pineal

Publications for High Grade Ependymoma

Articles related to High Grade Ependymoma:

(show top 50) (show all 78)
# Title Authors PMID Year
1
Tumors in the cauda equina: A SEER analysis of tumor types and predictors of outcome. 61
33222921 2020
2
RELA fusion-positive ependymoma accompanied by extensive desmoplasia: a case report. 61
32754892 2020
3
Novel nomograms predicting overall and cancer-specific survival of malignant ependymoma patients: a population-based study. 61
32972115 2020
4
Simultaneous cerebrospinal fluid and hematologic metastases in a high-grade ependymoma. 61
29770253 2018
5
Central nervous system tumors: Radiologic pathologic correlation and diagnostic approach. 61
25883479 2015
6
Response to temozolomide in supratentorial multifocal recurrence of malignant ependymoma. 61
21498732 2011
7
Cytologic findings in malignant ependymoma: a case report. 61
21053591 2010
8
Cachexia secondary to intracranial anaplastic (malignant) ependymoma in a boxer dog. 61
19796316 2009
9
Sacrococcygeal immature teratoma with malignant ependymoma component. 61
19492320 2009
10
A primary malignant ependymoma of the abdominal cavity: a case report and review of the literature. 61
19238432 2009
11
Giant cell ependymoma: a case report. 61
18006220 2008
12
Clinicopathological evaluation of immunohistochemical Ki-67 and endothelial nitric oxide synthase expression in intracranial ependymoma. 61
18682044 2008
13
Evaluation of intratumoral administration of tumor necrosis factor-alpha in patients with malignant glioma. 61
17195453 2006
14
Supratentorial malignant ependymoma in childhood: 16 years without relapse after hemispherectomy. 61
15095106 2005
15
Primitive neuroectodermal tumors in the central nervous system following cranial irradiation: a report of four cases. 61
12569608 2003
16
Molecular evidence of apoptotic death in malignant brain tumors including glioblastoma multiforme: upregulation of calpain and caspase-3. 61
12111801 2002
17
p14ARF protein (FL-132) immunoreactivity in intracranial ependymomas and its prognostic significance: an analysis of 103 cases. 61
11585252 2001
18
Antineuronal nuclei immunohistochemical staining patterns in childhood ependymomas. 61
11510923 2001
19
[Significance of expression and secretion of interleukin-8 in ependymal tumor]. 61
11798904 2001
20
Permanent low-activity (125)I seed placement for the treatment of pediatric brain tumors: preliminary experience. 61
11359113 2001
21
The local field in infratentorial ependymoma: does the entire posterior fossa need to be treated? 61
11172959 2001
22
Supratentorial giant cell ependymoma. 61
11220693 2001
23
The importance of surgery in supratentorial ependymomas. Long-term survival in a series of 23 cases. 61
10804053 2000
24
Optimal light dose for interstitial photodynamic therapy in treatment for malignant brain tumors. 61
11013384 2000
25
Phase II study of 6-thioguanine, procarbazine, dibromodulcitol, lomustine, and vincristine chemotherapy with radiotherapy for treating malignant glioma in children. 61
11302250 2000
26
[Anaplastic (malignant) ependymoma]. 61
11043175 2000
27
Molecular genetic analysis of ependymal tumors. NF2 mutations and chromosome 22q loss occur preferentially in intramedullary spinal ependymomas. 54
10433955 1999
28
Surgical outcome of intramedullary spinal cord ependymoma. 61
10352743 1999
29
Correlation of bcl-2, p53, and MIB-1 expression with ependymoma grade and subtype. 61
9619600 1998
30
Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children's Cancer Group. 61
9525716 1998
31
[Ectopic intraspinal extradural anaplastic ependymoma in an infant]. 61
9181020 1997
32
Hypofractionated stereotactic radiotherapy in the management of recurrent glioma. 61
9069312 1997
33
Molecular studies of an ependymoma-associated constitutional t(1;22)(p22;q11.2). 61
9465898 1997
34
Posterior fossa lateral ependymoma in childhood. 61
8988495 1996
35
Constitutional de novo t(1;22)(p22;q11.2) and ependymoma. 61
8603343 1996
36
[Intramedullary glioma. Postoperative MRI aspects]. 61
7629570 1995
37
Expression and localization of messenger RNA and protein for monocyte chemoattractant protein-1 in human malignant glioma. 61
8189261 1994
38
Malignant hemispheric tumors in childhood. 61
8397066 1993
39
Long-term follow-up in 39 patients with an ependymoma after surgery and irradiation. 61
1411924 1992
40
Primary malignant ependymoma of the lung. 61
1548954 1992
41
Infratentorial stereotactic biopsy. A review of 11 cases. 61
1295028 1992
42
Dissemination of supratentorial malignant gliomas via the cerebrospinal fluid in children. 61
1738457 1992
43
Identification of a germ-line mutation in the p53 gene in a patient with an intracranial ependymoma. 61
1679237 1991
44
Expression of platelet-derived growth factors, transforming growth factors, and the ros gene in a variety of primary human brain tumors. 61
1997889 1991
45
Efficacy of postoperative chemotherapy using cisplatin plus etoposide in young children with brain tumors. 61
1990253 1991
46
Expression of glial fibrillary acidic protein, vimentin, fibronectin, and N-myc oncoprotein in primary human brain tumor cell explants. 54
1668298 1991
47
Extracranial metastases of malignant ependymoma--case report. 61
1699151 1990
48
Localization and significance of peanut agglutinin-binding sites on ependymoma cells. 61
2360409 1990
49
Mapping of receptors for carbohydrate constituents of glycoconjugates in well-differentiated and malignant ependymomas: a glycohistochemical study. 61
2542827 1989
50
Lack of histopathological correlation of malignant ependymomas with postoperative survival. 61
2909684 1989

Variations for High Grade Ependymoma

Expression for High Grade Ependymoma

Search GEO for disease gene expression data for High Grade Ependymoma.

Pathways for High Grade Ependymoma

Pathways related to High Grade Ependymoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.47 YAP1 GFAP EGFR
2 11.34 TP53 PLA2G5 GFAP EGFR
3 11.18 TP53 IDH1 EGFR
4 10.97 TP53 NF2 MGMT EGFR

GO Terms for High Grade Ependymoma

Biological processes related to High Grade Ependymoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.26 TP53 EGFR
2 regulation of stem cell proliferation GO:0072091 9.16 YAP1 NF2
3 response to cobalamin GO:0033590 8.96 RELA EGFR
4 response to organic cyclic compound GO:0014070 8.92 RELA MGMT IDH1 EGFR

Sources for High Grade Ependymoma

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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