MCID: CNT045
MIFTS: 28

Central Nervous System Sarcoma

Categories: Cancer diseases, Neuronal diseases

Aliases & Classifications for Central Nervous System Sarcoma

MalaCards integrated aliases for Central Nervous System Sarcoma:

Name: Central Nervous System Sarcoma 12 15 73
Sarcoma of the Cns 12

Classifications:



External Ids:

Disease Ontology 12 DOID:2133
NCIt 50 C5153
UMLS 73 C1332892

Summaries for Central Nervous System Sarcoma

Disease Ontology : 12 A central nervous system cancer develops from transformed cells of mesenchymal origin located in brain and spine, has material basis in abnormally proliferating cells derives from mesoderm.

MalaCards based summary : Central Nervous System Sarcoma, also known as sarcoma of the cns, is related to kaposi sarcoma and central nervous system angiosarcoma. An important gene associated with Central Nervous System Sarcoma is SMARCA4 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily A, Member 4), and among its related pathways/superpathways are Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers and BRCA1 Pathway. The drugs Sunitinib and Angiogenesis Inhibitors have been mentioned in the context of this disorder. Affiliated tissues include brain, skin and liver, and related phenotypes are hematopoietic system and immune system

Related Diseases for Central Nervous System Sarcoma

Diseases related to Central Nervous System Sarcoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 37)
# Related Disease Score Top Affiliating Genes
1 kaposi sarcoma 11.2
2 central nervous system angiosarcoma 11.1
3 rhabdoid cancer 10.1 SMARCA4 SMARCB1
4 tumor predisposition syndrome 10.1 SMARCA4 SMARCB1
5 lymphoplasmacyte-rich meningioma 10.0 CD163 CD68
6 spleen cancer 10.0 CD163 CD68
7 atypical teratoid rhabdoid tumor 10.0 SMARCA4 SMARCB1
8 petroclival meningioma 10.0 CD163 CD68
9 borjeson-forssman-lehmann syndrome 10.0 SMARCA4 SMARCB1
10 photokeratitis 10.0 CD163 CD68
11 skin sarcoma 10.0 CD163 CD68
12 coffin-siris syndrome 1 10.0 SMARCA4 SMARCB1
13 enterobiasis 10.0 CD163 CD68
14 connective tissue cancer 10.0 CD68 SMARCB1
15 hypertrichosis 10.0 SMARCA4 SMARCB1
16 cardiomyopathy, infantile histiocytoid 9.9 CD68 LYZ
17 malignant giant cell tumor of soft parts 9.9 CD68 LYZ
18 liver sarcoma 9.9 CD68 LYZ
19 granulomatous myositis 9.9 CD68 LYZ
20 trigeminal nerve neoplasm 9.9 CD68 LYZ
21 neurilemmoma of the fifth cranial nerve 9.9 CD68 LYZ
22 acute salpingitis 9.9 CD68 LYZ
23 aleukemic leukemia cutis 9.9 CD68 LYZ
24 ulcerative stomatitis 9.9 CD68 LYZ
25 xanthogranulomatous pyelonephritis 9.9 CD68 LYZ
26 dermis tumor 9.9 CD163 LYZ
27 congenital epulis 9.9 CD68 LYZ
28 autosomal dominant non-syndromic intellectual disability 9.9 SMARCA4 SMARCB1
29 non-langerhans-cell histiocytosis 9.9 CD163 LYZ
30 sarcoma 9.9
31 histiocytic and dendritic cell cancer 9.9 CD163 LYZ
32 xanthogranulomatous cholecystitis 9.9 CD68 LYZ
33 synovitis 9.9 CD163 CD68
34 lymphatic system disease 9.9 CD68 LYZ
35 meningioma, familial 9.8 CD68 SMARCB1
36 adult xanthogranuloma 9.7 CD163 CD68 LYZ
37 reticulohistiocytic granuloma 9.7 CD163 CD68 LYZ

Graphical network of the top 20 diseases related to Central Nervous System Sarcoma:



Diseases related to Central Nervous System Sarcoma

Symptoms & Phenotypes for Central Nervous System Sarcoma

MGI Mouse Phenotypes related to Central Nervous System Sarcoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.35 CD163 CD68 LYZ SMARCA4 SMARCB1
2 immune system MP:0005387 9.02 CD163 CD68 LYZ SMARCA4 SMARCB1

Drugs & Therapeutics for Central Nervous System Sarcoma

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
2 Angiogenesis Inhibitors Phase 2
3 Angiogenesis Modulating Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Sunitinib in Sarcomas of the Central Nervous System Not yet recruiting NCT03641326 Phase 2 Sunitinib
2 Rare CNS Tumors Outcomes &Risk Recruiting NCT03251989

Search NIH Clinical Center for Central Nervous System Sarcoma

Genetic Tests for Central Nervous System Sarcoma

Anatomical Context for Central Nervous System Sarcoma

MalaCards organs/tissues related to Central Nervous System Sarcoma:

41
Brain, Skin, Liver, Spleen

The Foundational Model of Anatomy Ontology organs/tissues related to Central Nervous System Sarcoma:

19
Brain

Publications for Central Nervous System Sarcoma

Articles related to Central Nervous System Sarcoma:

# Title Authors Year
1
Association of central nervous system sarcoma with familial polyposis coli. ( 386305 )
1978

Variations for Central Nervous System Sarcoma

Expression for Central Nervous System Sarcoma

Search GEO for disease gene expression data for Central Nervous System Sarcoma.

Pathways for Central Nervous System Sarcoma

GO Terms for Central Nervous System Sarcoma

Cellular components related to Central Nervous System Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 SWI/SNF complex GO:0016514 9.16 SMARCA4 SMARCB1
2 nBAF complex GO:0071565 8.96 SMARCA4 SMARCB1
3 npBAF complex GO:0071564 8.62 SMARCA4 SMARCB1

Biological processes related to Central Nervous System Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of DNA-binding transcription factor activity GO:0051091 9.43 SMARCA4 SMARCB1
2 chromatin remodeling GO:0006338 9.4 SMARCA4 SMARCB1
3 ATP-dependent chromatin remodeling GO:0043044 9.37 SMARCA4 SMARCB1
4 nucleosome disassembly GO:0006337 9.32 SMARCA4 SMARCB1
5 positive regulation by host of viral transcription GO:0043923 9.26 SMARCA4 SMARCB1
6 positive regulation of transcription of nucleolar large rRNA by RNA polymerase I GO:1901838 9.16 SMARCA4 SMARCB1
7 RNA polymerase I preinitiation complex assembly GO:0001188 8.96 SMARCA4 SMARCB1
8 positive regulation of glucose mediated signaling pathway GO:1902661 8.62 SMARCA4 SMARCB1

Molecular functions related to Central Nervous System Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 RNA polymerase II distal enhancer sequence-specific DNA binding GO:0000980 9.32 SMARCA4 SMARCB1
2 p53 binding GO:0002039 9.26 SMARCA4 SMARCB1
3 nucleosomal DNA binding GO:0031492 9.16 SMARCA4 SMARCB1
4 Tat protein binding GO:0030957 8.96 SMARCA4 SMARCB1
5 RNA polymerase I CORE element sequence-specific DNA binding GO:0001164 8.62 SMARCA4 SMARCB1

Sources for Central Nervous System Sarcoma

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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