MCID: PST113
MIFTS: 27

Posterior Fossa Ependymoma

Categories: Bone diseases, Cancer diseases, Neuronal diseases

Aliases & Classifications for Posterior Fossa Ependymoma

MalaCards integrated aliases for Posterior Fossa Ependymoma:

Name: Posterior Fossa Ependymoma 12

Classifications:



External Ids:

Disease Ontology 12 DOID:0080889

Summaries for Posterior Fossa Ependymoma

Disease Ontology : 12 A high grade ependymoma that is located within the posterior fossa.

MalaCards based summary : Posterior Fossa Ependymoma is related to benign ependymoma and cellular ependymoma. An important gene associated with Posterior Fossa Ependymoma is EZHIP (EZH Inhibitory Protein), and among its related pathways/superpathways are Chromatin Regulation / Acetylation and TGF-beta signaling pathway (KEGG). The drugs Sargramostim and Trastuzumab have been mentioned in the context of this disorder. Affiliated tissues include brain and pineal, and related phenotypes are endocrine/exocrine gland and neoplasm

Related Diseases for Posterior Fossa Ependymoma

Graphical network of the top 20 diseases related to Posterior Fossa Ependymoma:



Diseases related to Posterior Fossa Ependymoma

Symptoms & Phenotypes for Posterior Fossa Ependymoma

MGI Mouse Phenotypes related to Posterior Fossa Ependymoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.55 AMH CDKN2A EZH2 INHBB TET2
2 neoplasm MP:0002006 9.35 AMH CDKN2A EZH2 SUZ12 TET2
3 no phenotypic analysis MP:0003012 8.92 AMH CDKN2A EZH2 TET2

Drugs & Therapeutics for Posterior Fossa Ependymoma

Drugs for Posterior Fossa Ependymoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 8)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sargramostim Approved, Investigational Phase 1 123774-72-1, 83869-56-1
2
Trastuzumab Approved, Investigational Phase 1 180288-69-1 9903
3
Decitabine Approved, Investigational Phase 1 2353-33-5 451668
4
Azacitidine Approved, Investigational Phase 1 320-67-2 9444
5
Molgramostim Investigational Phase 1 99283-10-0
6 Antineoplastic Agents, Immunological Phase 1
7 Immunologic Factors Phase 1
8 Antimetabolites Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Pilot Study of the Effect of Trastuzumab and GM-CSF on Children With Recurrent Ependymoma Recruiting NCT02774421 Phase 1 Trastuzumab after SubQ GM-CSF;Trastuzumab in combination with SubQ GM-CSF
2 Infusion of 5-Azacytidine (5-AZA) Into the Fourth Ventricle or Resection Cavity in Children and Adults With Recurrent Posterior Fossa Ependymoma: A Phase I Study Recruiting NCT03572530 Phase 1 5-Azacytidine (5-AZA) group 1;5-Azacytidine (5-AZA) group 2;5-Azacytidine (5-AZA) group 3
3 Infusion of 5-Azacytidine (5-AZA) Into the Fourth Ventricle or Resection Cavity in Children With Recurrent Posterior Fossa Ependymoma: A Pilot Study Completed NCT02940483 Early Phase 1 5-Azacytidine

Search NIH Clinical Center for Posterior Fossa Ependymoma

Genetic Tests for Posterior Fossa Ependymoma

Anatomical Context for Posterior Fossa Ependymoma

MalaCards organs/tissues related to Posterior Fossa Ependymoma:

40
Brain, Pineal

Publications for Posterior Fossa Ependymoma

Articles related to Posterior Fossa Ependymoma:

(show top 50) (show all 81)
# Title Authors PMID Year
1
Medulloblastoma: Clinical presentation. 61
31494131 2021
2
Spatial Agreement of Brainstem Dose Distributions Depending on Biological Model in Proton Therapy for Pediatric Brain Tumors. 61
33490724 2021
3
Posterior fossa ependymoma with preoperative cerebrospinal metastases: a case report with literature review. 61
33300386 2020
4
Eosinophilic globules in a classic ependymoma: evidence of a possible secretory role. 61
33148106 2020
5
cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors. 61
32502305 2020
6
Radiology Profile as a Potential Instrument to Differentiate Between Posterior Fossa Ependymoma (PF-EPN) Group A and B. 61
32428725 2020
7
CN133, a Novel Brain-Penetrating Histone Deacetylase Inhibitor, Hampers Tumor Growth in Patient-Derived Pediatric Posterior Fossa Ependymoma Models. 61
32708733 2020
8
Radiation-induced Supratentorial Osteosarcoma Following Curative Treatment of Infratentorial Ependymoma in a Child. 61
33531953 2020
9
Familial Cerebral Cavernous Malformation Syndrome with Concomitant Fourth Ventricular Ependymoma: True Association or Mere Coincidence? 61
32434131 2020
10
Is H3K27me3 status really a strong prognostic indicator for pediatric posterior fossa ependymomas? A single surgeon, single center experience. 61
32025869 2020
11
Familial intracranial ependymoma mimicking an extra-lesion: A case report and review of the literature. 61
32146015 2020
12
TET2 promotor methylation and TET2 protein expression in pediatric posterior fossa ependymoma. 61
31777116 2020
13
Adult outcome after treatment of pediatric posterior fossa ependymoma: long-term follow-up of a single consecutive institutional series of 22 patients with more than 5 years of survival. 61
32217795 2020
14
Molecular characterization of histopathological ependymoma variants. 61
31679042 2020
15
Chondro-Osseous Metaplasia in Ependymoma: A Rare Histopathological Finding. 61
32455040 2020
16
Characterization of global 5-hydroxymethylcytosine in pediatric posterior fossa ependymoma. 61
31992357 2020
17
MYCN amplification drives an aggressive form of spinal ependymoma. 61
31414211 2019
18
Improved risk-stratification for posterior fossa ependymoma of childhood considering clinical, histological and genetic features - a retrospective analysis of the HIT ependymoma trial cohort. 61
31727173 2019
19
Molecular grouping and outcomes of young children with newly diagnosed ependymoma treated on the multi-institutional SJYC07 trial. 61
30976811 2019
20
Radiation doses to brain substructures associated with cognition in radiotherapy of pediatric brain tumors. 61
31271084 2019
21
Newly Diagnosed Metastatic Intracranial Ependymoma in Children: Frequency, Molecular Characteristics, Treatment, and Outcome in the Prospective HIT Series. 61
30850560 2019
22
EZHIP/CXorf67 mimics K27M mutated oncohistones and functions as an intrinsic inhibitor of PRC2 function in aggressive posterior fossa ependymoma. 61
30923826 2019
23
The European Society of Paediatric Oncology Ependymoma-II program Core-Plus model: Development and initial implementation of a cognitive test protocol for an international brain tumour trial. 61
31182404 2019
24
Survival and functional outcomes of molecularly defined childhood posterior fossa ependymoma: Cure at a cost. 61
30768777 2019
25
Surgical Management of Isolated Fourth Ventricular Hydrocephalus Associated with Injury to the Guillain-Mollaret Triangle. 61
30368016 2019
26
Long-Term Supratentorial Radiologic Effects of Surgery and Local Radiation in Children with Infratentorial Ependymoma. 61
30448581 2019
27
Infusion of 5-Azacytidine (5-AZA) into the fourth ventricle or resection cavity in children with recurrent posterior Fossa Ependymoma: a pilot clinical trial. 61
30460634 2019
28
Significance of molecular classification of ependymomas: C11orf95-RELA fusion-negative supratentorial ependymomas are a heterogeneous group of tumors. 61
30514397 2018
29
Prognostic and microRNA profile analysis for CD44 positive expression pediatric posterior fossa ependymoma. 61
29704232 2018
30
Sulfonylurea receptor 1 expression is variable in adult and pediatric brain tumors. 61
30079884 2018
31
Heterogeneity within the PF-EPN-B ependymoma subgroup. 61
30019219 2018
32
Impact of radiation therapy and extent of resection for ependymoma in young children: A population-based study. 61
29115718 2018
33
Therapeutic targeting of ependymoma as informed by oncogenic enhancer profiling. 61
29258295 2018
34
p16 Loss and E2F/cell cycle deregulation in infant posterior fossa ependymoma. 61
28548702 2017
35
Giant Cell Ependymoma of Lateral Ventricle: Case Report, Literature Review, and Analysis of Prognostic Factors and Genetic Profile. 61
28943417 2017
36
Immunohistochemical analysis of H3K27me3 demonstrates global reduction in group-A childhood posterior fossa ependymoma and is a powerful predictor of outcome. 61
28733933 2017
37
Four-Hand Suction-Irrigation Technique Leads to Gross Total Resection and Long-Term Progression-Free Survival in Fourth Ventricular Ependymoma. 61
28804042 2017
38
Telomerase activation in posterior fossa group A ependymomas is associated with dismal prognosis and chromosome 1q gain. 61
28371821 2017
39
H3 K27M mutations are extremely rare in posterior fossa group A ependymoma. 61
28623522 2017
40
Treatment implications of posterior fossa ependymoma subgroups. 61
27846874 2016
41
Assessing 'second-look' tumour resectability in childhood posterior fossa ependymoma-a centralised review panel and staging tool for future studies. 61
27585992 2016
42
Chromosome 1q gain and tenascin-C expression are candidate markers to define different risk groups in pediatric posterior fossa ependymoma. 61
27550150 2016
43
Quantitative imaging analysis of posterior fossa ependymoma location in children. 61
27117266 2016
44
Therapeutic Impact of Cytoreductive Surgery and Irradiation of Posterior Fossa Ependymoma in the Molecular Era: A Retrospective Multicohort Analysis. 61
27269943 2016
45
Treatment and survival of supratentorial and posterior fossa ependymomas in adults. 61
26810473 2016
46
The Similarities and Differences between Intracranial and Spinal Ependymomas : A Review from a Genetic Research Perspective. 61
26962412 2016
47
Posterior fossa ependymoma: current insights. 61
26351223 2015
48
Posterior fossa ependymoma in childhood: 60 years event-free survival after partial resection—a case report. 61
26047947 2015
49
EZH2 expression is a prognostic factor in childhood intracranial ependymoma: a Canadian Pediatric Brain Tumor Consortium study. 61
25586788 2015
50
The role of resection alone in select children with intracranial ependymoma: the Canadian Pediatric Brain Tumour Consortium experience. 61
25391979 2015

Variations for Posterior Fossa Ependymoma

Expression for Posterior Fossa Ependymoma

Search GEO for disease gene expression data for Posterior Fossa Ependymoma.

Pathways for Posterior Fossa Ependymoma

Pathways related to Posterior Fossa Ependymoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.54 TET2 SUZ12 EZH2
2 11.23 INHBB AMH
3 10.95 TET2 EZH2
4 9.85 SUZ12 EZH2

GO Terms for Posterior Fossa Ependymoma

Cellular components related to Posterior Fossa Ependymoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 ESC/E(Z) complex GO:0035098 8.62 SUZ12 EZH2

Biological processes related to Posterior Fossa Ependymoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 chromatin organization GO:0006325 9.33 TET2 SUZ12 EZH2
2 negative regulation of G1/S transition of mitotic cell cycle GO:2000134 9.32 EZH2 CDKN2A
3 negative regulation of gene expression, epigenetic GO:0045814 9.26 SUZ12 EZH2
4 negative regulation of G0 to G1 transition GO:0070317 8.96 SUZ12 EZH2
5 histone H3-K27 trimethylation GO:0098532 8.62 SUZ12 EZH2

Molecular functions related to Posterior Fossa Ependymoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 chromatin DNA binding GO:0031490 9.26 SUZ12 EZH2
2 RNA polymerase II transcription corepressor binding GO:0001226 9.16 SUZ12 EZH2
3 histone methyltransferase activity GO:0042054 8.96 SUZ12 EZH2
4 histone methyltransferase activity (H3-K27 specific) GO:0046976 8.62 SUZ12 EZH2

Sources for Posterior Fossa 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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