MCID: CHR033
MIFTS: 35

Chordoid Glioma

Categories: Neuronal diseases, Rare diseases, Cancer diseases

Aliases & Classifications for Chordoid Glioma

MalaCards integrated aliases for Chordoid Glioma:

Name: Chordoid Glioma 12 59 15
Chordoid Glioma of the Third Ventricle 73

Characteristics:

Orphanet epidemiological data:

59
chordoid glioma
Age of onset: Adult; Age of death: adult;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:3774
NCIt 50 C5592
SNOMED-CT 68 128789002
Orphanet 59 ORPHA251674
ICD10 via Orphanet 34 C71.9
UMLS 73 C1322252

Summaries for Chordoid Glioma

MalaCards based summary : Chordoid Glioma, also known as chordoid glioma of the third ventricle, is related to third ventricle chordoid glioma and glioma. An important gene associated with Chordoid Glioma is GFAP (Glial Fibrillary Acidic Protein), and among its related pathways/superpathways are Development EGFR signaling pathway and Neuroscience. The drugs Dabrafenib and Trametinib have been mentioned in the context of this disorder. Affiliated tissues include brain, thyroid and hypothalamus, and related phenotypes are Increased proliferation and neoplasm

Related Diseases for Chordoid Glioma

Diseases related to Chordoid Glioma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 166)
# Related Disease Score Top Affiliating Genes
1 third ventricle chordoid glioma 12.4
2 glioma 10.8
3 localized hypertrophic neuropathy 10.7 MUC1 S100B
4 sclerosing perineurioma 10.7 MUC1 S100B
5 reticular perineurioma 10.6 MUC1 S100B
6 non-functioning pituitary adenoma 10.6 MUC1 S100B
7 epithelioid malignant peripheral nerve sheath tumor 10.6 MUC1 S100B
8 ossifying fibromyxoid tumor 10.6 MUC1 S100B
9 eccrine porocarcinoma 10.6 MUC1 S100B
10 aggressive digital papillary adenocarcinoma 10.6 MUC1 S100B
11 monophasic synovial sarcoma 10.6 MUC1 S100B
12 pleomorphic liposarcoma 10.6 MUC1 S100B
13 gemistocytic astrocytoma 10.6 GFAP S100B
14 dysembryoplastic neuroepithelial tumor 10.6 GFAP S100B
15 spindle cell sarcoma 10.6 MUC1 S100B
16 functioning pituitary adenoma 10.6 MUC1 S100B
17 melanotic neuroectodermal tumor 10.5 GFAP S100B
18 meningeal melanocytoma 10.5 MUC1 S100B
19 angiocentric glioma 10.5 GFAP S100B
20 cutaneous leiomyosarcoma 10.5 GFAP MUC1
21 pineocytoma 10.5 GFAP S100B
22 dendritic cell tumor 10.5 MUC1 S100B
23 parachordoma 10.5 GFAP MUC1
24 hemangiopericytoma, malignant 10.5 MUC1 S100B
25 clear cell meningioma 10.5 GFAP MUC1
26 papillary ependymoma 10.5 GFAP S100B
27 polymorphous low-grade adenocarcinoma 10.5 MUC1 S100B
28 subependymal giant cell astrocytoma 10.5 GFAP S100B
29 malignant giant cell tumor 10.5 S100B TP53
30 intraneural perineurioma 10.5 MUC1 S100B
31 granular cell tumor 10.5 GFAP S100B
32 traumatic brain injury 10.5 GFAP S100B
33 microglandular adenosis 10.5 S100B TP53
34 malignant fibroxanthoma 10.5 MUC1 S100B
35 pleuropulmonary blastoma 10.4 S100B TP53
36 frontal convexity meningioma 10.4 GFAP TP53
37 gliofibroma 10.4 GFAP TP53
38 protoplasmic astrocytoma 10.4 GFAP TP53
39 pilomatrixoma 10.4 MUC1 S100B
40 dedifferentiated liposarcoma 10.4 MUC1 TP53
41 cellular ependymoma 10.4 GFAP TP53
42 biliary papillomatosis 10.4 MUC1 TP53
43 embryonal sarcoma 10.4 S100B TP53
44 central nervous system primitive neuroectodermal neoplasm 10.4 GFAP TP53
45 cerebral neuroblastoma 10.4 GFAP TP53
46 mixed glioma 10.4 GFAP TP53
47 myoepithelioma 10.4 GFAP S100B
48 ependymoblastoma 10.4 GFAP TP53
49 cerebral ventricle cancer 10.4 GFAP TP53
50 cerebrum cancer 10.4 GFAP TP53

Graphical network of the top 20 diseases related to Chordoid Glioma:



Diseases related to Chordoid Glioma

Symptoms & Phenotypes for Chordoid Glioma

GenomeRNAi Phenotypes related to Chordoid Glioma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased proliferation GR00094-A 8.62 NF2 TP53

MGI Mouse Phenotypes related to Chordoid Glioma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 neoplasm MP:0002006 8.92 NF2 NKX2-1 PRKCA TP53

Drugs & Therapeutics for Chordoid Glioma

Drugs for Chordoid Glioma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dabrafenib Approved, Investigational Phase 2 1195765-45-7 44462760 44516822
2
Trametinib Approved Phase 2 871700-17-3 11707110
3 Protein Kinase Inhibitors Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase II Pediatric Study With Dabrafenib in Combination With Trametinib in Patients With HGG and LGG Recruiting NCT02684058 Phase 2 dabrafenib;trametinib;Carboplatin with vincristine

Search NIH Clinical Center for Chordoid Glioma

Genetic Tests for Chordoid Glioma

Anatomical Context for Chordoid Glioma

MalaCards organs/tissues related to Chordoid Glioma:

41
Brain, Thyroid, Hypothalamus, Testes

Publications for Chordoid Glioma

Articles related to Chordoid Glioma:

(show top 50) (show all 71)
# Title Authors Year
1
A recurrent kinase domain mutation in PRKCA defines chordoid glioma of the third ventricle. ( 29476136 )
2018
2
The trans-laminar terminalis approach reduces mortalities associated with chordoid glioma resections: A case report and a review of 20a8^years of literature. ( 29066234 )
2018
3
Gross Total Resection of Chordoid Glioma of the Third Ventricle via Anterior Interhemispheric Transcallosal Transforaminal Approach at Two Stages. ( 29588902 )
2018
4
Letter to the editor regarding "The trans-laminar terminalis approach reduces mortalities associated with chordoid glioma resections: A case report and a review of 20a8^years of literature". ( 29605280 )
2018
5
Chordoid Glioma: A Neoplasm Found in the Anterior Part of the Third Ventricle. ( 28708638 )
2017
6
Management in chordoid glioma: Avoiding the pitfalls in this rare and challenging entity. ( 28681757 )
2017
7
Chordoid glioma of the third ventricle: report of a rapidly progressive case. ( 28315998 )
2017
8
Corrigendum to "The radiological findings of chordoid glioma: report of two cases, one case with MR spectroscopy" [Clin Imaging 39 (2015) 1086-1089]. ( 27133706 )
2016
9
Chordoid Glioma of Third Ventricle With an Epidermoid Cyst: Coexistence or Common Histogenesis? ( 27194402 )
2016
10
Neurogenic fever in a patient with a chordoid glioma. ( 27856533 )
2016
11
Chordoid glioma of the third ventricle: a patient presenting with SIADH and a review of this rare tumor. ( 26879322 )
2016
12
Expanded Endoscopic Transnasal Approach to the Chordoid Glioma of the Third Ventricle : The First Case Ever Reported. ( 27847580 )
2016
13
Chordoid Glioma with Intraventricular Dissemination: A Case Report with Perfusion MR Imaging Features. ( 26798226 )
2016
14
Chordoid glioma: A new paradigm of hypothalamic dysfunction? ( 27798757 )
2016
15
Chordoid glioma: a rare radiologically, histologically, and clinically mystifying lesion. ( 26018908 )
2015
16
Suprasellar chordoid neoplasm with expression of thyroid transcription factor 1: evidence that chordoid glioma of the third ventricle and pituicytoma may form part of a spectrum of lineage-related tumors of the basal forebrain. ( 25934249 )
2015
17
Chordoid glioma. ( 26668411 )
2015
18
Chordoid glioma: Case report and review of the literature. ( 25648470 )
2015
19
Chordoid glioma of the third ventricle. ( 26543291 )
2015
20
The radiological findings of chordoid glioma: report of two cases, one case with MR spectroscopy. ( 26275847 )
2015
21
Expression of thyroid transcription factor 1 in a chordoid glioma. ( 25248954 )
2014
22
Neuro-Ophthalmologic Features of Chordoid Glioma. ( 24113763 )
2013
23
Therapeutic approach to chordoid glioma of the third ventricle. ( 23615418 )
2013
24
Chordoid glioma of the third ventricle: four cases including one case with papillary features. ( 22716306 )
2013
25
Chordoid glioma: a rare suprasellar mass. ( 22644806 )
2012
26
Chordoid glioma of the third ventricle. ( 22754136 )
2012
27
Cytology of chordoid glioma of the third ventricle. ( 22246938 )
2012
28
CD99 is expressed in chordoid glioma and suggests ependymal origin. ( 22102064 )
2012
29
Chordoid glioma in suprasellar location with extension into the third ventricle: smear preparation morphology of a rare tumor. ( 22246933 )
2012
30
Rarest of the rare: Chordoid glioma infiltrating the optic chiasm. ( 21697970 )
2011
31
Chordoid glioma: a case report and literature review. ( 21192197 )
2011
32
Chordoid glioma of the third ventricle: a case report with review of literature. ( 21743188 )
2011
33
Chordoid glioma: imaging pearls of a unique third ventricular tumor. ( 21059517 )
2010
34
Chordoid glioma: ten years of a low-grade tumor with high morbidity. ( 20808539 )
2010
35
Chordoid glioma : a case report of unusual location and neuroradiological characteristics. ( 20717514 )
2010
36
Chordoid glioma with calcification and neurofilament expression: case report and review of the literature. ( 18262609 )
2009
37
Chordoid glioma with calcification and neurofilament expression: case report and review of the literature (Iwami et al. Surg Neurol 2008;10.1016/j.surneu.2007.07.032). ( 19329155 )
2009
38
Diabetes insipidus, panhypopituitarism, and severe mental status deterioration in a patient with chordoid glioma: case report and literature review. ( 19364693 )
2009
39
Chordoid glioma of the third ventricle: a report of two cases, one with ultrastructural findings. ( 18498285 )
2009
40
Chordoid glioma: a case report and molecular characterization of five cases. ( 18652591 )
2009
41
Chordoid glioma of the third ventricle attached to the optic chiasm. Successful removal through a trans-lamina terminalis approach. ( 18597929 )
2008
42
Chordoid glioma of the third ventricle. ( 18936876 )
2008
43
Chordoid glioma: report of two rare examples with unusual features. ( 18246456 )
2008
44
An incidentally detected third ventricle chordoid glioma. ( 18040120 )
2007
45
Extraventricular unusual glioma in a child with extensive myxoid change resembling chordoid glioma. ( 17965223 )
2007
46
Test and teach. A recurrent third ventricular brain tumour. Diagnosis: Chordoid glioma of the third ventricle. ( 16753749 )
2006
47
Third ventricular chordoid glioma with unusual aggressive behavior. ( 17185888 )
2006
48
Origin of chordoid glioma of the third ventricle. ( 16594739 )
2006
49
Chordoid glioma of the third ventricle. ( 16568345 )
2006
50
Chordoid glioma of the third ventricle: Report of a case with cytologic features and utility during intraoperative consultation. ( 17152286 )
2006

Variations for Chordoid Glioma

Expression for Chordoid Glioma

Search GEO for disease gene expression data for Chordoid Glioma.

Pathways for Chordoid Glioma

Pathways related to Chordoid Glioma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.82 MUC1 PRKCA TP53
2 11.46 GFAP NF2 NKX2-1 S100B
3
Show member pathways
11.35 PRKCA TP53
4 11.21 GFAP PRKCA TP53

GO Terms for Chordoid Glioma

Biological processes related to Chordoid Glioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hippocampus development GO:0021766 9.26 NF2 NKX2-1
2 DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest GO:0006977 9.16 MUC1 TP53
3 long-term synaptic potentiation GO:0060291 8.96 GFAP S100B
4 DNA damage response, signal transduction by p53 class mediator resulting in transcription of p21 class mediator GO:0006978 8.62 MUC1 TP53

Molecular functions related to Chordoid Glioma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 enzyme binding GO:0019899 8.8 NKX2-1 PRKCA TP53

Sources for Chordoid Glioma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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