MCID: CNT031
MIFTS: 31

Central Epithelioid Sarcoma

Categories: Cancer diseases, Rare diseases

Aliases & Classifications for Central Epithelioid Sarcoma

MalaCards integrated aliases for Central Epithelioid Sarcoma:

Name: Central Epithelioid Sarcoma 12 15
Proximal-Type Epithelioid Sarcoma 71

Classifications:



External Ids:

Disease Ontology 12 DOID:7492
NCIt 49 C27472
UMLS 71 C1335563

Summaries for Central Epithelioid Sarcoma

MalaCards based summary : Central Epithelioid Sarcoma, also known as proximal-type epithelioid sarcoma, is related to central nervous system angiosarcoma and vulvar sarcoma. An important gene associated with Central Epithelioid Sarcoma is SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1), and among its related pathways/superpathways are Cytoskeletal Signaling and Keratinization. Affiliated tissues include liver, lung and cervix, and related phenotype is Reduced mammosphere formation.

Related Diseases for Central Epithelioid Sarcoma

Diseases in the Epithelioid Sarcoma family:

Central Epithelioid Sarcoma Adult Epithelioid Sarcoma

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

(show top 50) (show all 398)
# Related Disease Score Top Affiliating Genes
1 central nervous system angiosarcoma 10.6 SMARCB1 CD34
2 vulvar sarcoma 10.6 SMARCB1 CD34
3 glomangioma 10.6 VIM CD34
4 retroperitoneal leiomyosarcoma 10.6 SMARCB1 CD34
5 alk+ histiocytosis 10.6 CD207 CD1A
6 melanotic neurilemmoma 10.5 SMARCB1 CD34
7 glomangiomyoma 10.5 VIM CD34
8 pediatric liposarcoma 10.5 VIM CD34
9 mesenchymoma 10.5 MB CD34
10 epithelioid malignant peripheral nerve sheath tumor 10.5 SMARCB1 CD34
11 verruciform xanthoma of skin 10.5 CD1E CD1A
12 retroperitoneal sarcoma 10.5 SMARCB1 CD34
13 gallbladder leiomyosarcoma 10.5 MB CD34
14 lung leiomyosarcoma 10.5 KRT5 CD34
15 multifocal motor neuropathy 10.5 CD1E CD1A
16 spermatic cord cancer 10.4 KRT5 CD34
17 angiolipoma 10.4 VIM CD34
18 ovarian solid teratoma 10.4 TNFRSF8 CD34
19 kidney rhabdoid cancer 10.4 SMARCB1 MB
20 esophagus sarcoma 10.4 KRT5 CD34
21 multicentric reticulohistiocytosis 10.4 VIM CD1E
22 interstitial pneumonitis, desquamative, familial 10.4 CD1E CD1A
23 thymus adenocarcinoma 10.4 KRT5 KRT20
24 testicular fibroma 10.4 KRT5 CD34
25 spindle cell lipoma 10.4 VIM CD34
26 central nervous system fibrosarcoma 10.4 KRT7 CD34
27 liposarcoma of bone 10.4 TNFRSF8 CD34
28 breast myofibroblastoma 10.4 MME CD34
29 breast hemangiopericytoma 10.4 KRT7 CD34
30 neurilemmoma 10.4 VIM SMARCB1 CD34
31 nephrogenic adenofibroma 10.4 KRT7 CD34
32 pacinian tumor 10.4 MME CD34
33 urethral villous adenoma 10.4 KRT7 KRT20
34 malignant mesenchymoma 10.4 MB CD34
35 pancreatic serous cystadenocarcinoma 10.4 KRT7 KRT20
36 intrahepatic bile duct adenoma 10.4 KRT7 KRT20
37 seminal vesicle adenocarcinoma 10.4 KRT7 KRT20
38 liver fibroma 10.4 VIM KRT5
39 gastric signet ring cell adenocarcinoma 10.3 KRT7 KRT20
40 lichen nitidus 10.3 CD1E CD1B CD1A
41 cortical thymoma 10.3 CD1E CD1B CD1A
42 breast leiomyoma 10.3 MME CD34
43 atrophic vulva 10.3 KRT7 KRT20
44 dendritic cell thymoma 10.3 CD1E CD1B CD1A
45 bladder lymphoma 10.3 MME KRT20
46 urinary bladder villous adenoma 10.3 KRT7 KRT20
47 cystitis cystica 10.3 KRT7 KRT20
48 histiocytoid hemangioma 10.3 TNFRSF8 CD34
49 bile duct cystadenocarcinoma 10.3 KRT7 KRT20
50 inflammatory liposarcoma 10.3 MME CD34

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



Diseases related to Central Epithelioid Sarcoma

Symptoms & Phenotypes for Central Epithelioid Sarcoma

GenomeRNAi Phenotypes related to Central Epithelioid Sarcoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.17 CD1A CD1D CD34 KRT18 KRT7 SMARCB1

Drugs & Therapeutics for Central Epithelioid Sarcoma

Search Clinical Trials , NIH Clinical Center for Central Epithelioid Sarcoma

Genetic Tests for Central Epithelioid Sarcoma

Anatomical Context for Central Epithelioid Sarcoma

MalaCards organs/tissues related to Central Epithelioid Sarcoma:

40
Liver, Lung, Cervix, Appendix

Publications for Central Epithelioid Sarcoma

Articles related to Central Epithelioid Sarcoma:

(show top 50) (show all 75)
# Title Authors PMID Year
1
Epithelioid sarcoma of paratesticular region: A case report of an unusual case. 61
30971563 2019
2
Proximal-Type Epithelioid Sarcoma in Skull Base: a Pathological Diagnosis Challenge with Other Intracranial Tumors. 61
29076075 2019
3
Ultrasonography findings of proximal-type epithelioid sarcoma of the vulva: A case report. 61
30345044 2018
4
Proximal-type epithelioid sarcoma of the oral cavity: a case report and literature review. 61
30087080 2018
5
Proximal-type epithelioid sarcoma of the vulva: Cytopathological diagnosis of a rare neoplasm. 61
29683530 2018
6
Immunohistochemical Biomarkers of Mesenchymal Neoplasms in Endocrine Organs: Diagnostic Pitfalls and Recent Discoveries. 61
29340997 2018
7
Expression of CD34 and β-Catenin in Malignant Rhabdoid Tumor of the Liver Mimicking Proximal-Type Epithelioid Sarcoma. 61
28683521 2018
8
Clinicopathological analysis of myxoid proximal-type epithelioid sarcoma. 61
29068073 2018
9
Myoepithelial carcinoma or epithelioid sarcoma - A rare diagnosis with poor prognosis. A case report and review of literature. 61
30053614 2018
10
Proximal-Type Epithelioid Sarcoma: Report of an Unusual Case in the Uterine Cervix. 61
28381140 2017
11
Secondary EWSR1 gene abnormalities in SMARCB1-deficient tumors with 22q11-12 regional deletions: Potential pitfalls in interpreting EWSR1 FISH results. 61
27218413 2016
12
Osteoclast-rich, proximal-type epithelioid sarcoma: clinicopathologic features of 3 unusual cases expanding the histomorphological spectrum. 61
27040929 2016
13
Proximal-type epithelioid sarcoma: a new case report and literature review. 61
27800093 2016
14
Fine-needle-aspiration cytology of a proximal type epithelioid sarcoma: A case report. 61
26138930 2015
15
Malignant extra-renal rhabdoid tumor with unusual presentation: A report of two cases. 61
26881557 2015
16
Successful Treatment with Pazopanib for Multiple Lung Metastases of Inguinal Epithelioid Sarcoma: A Case Report. 61
26500539 2015
17
SMARCB1-deficient Vulvar Neoplasms: A Clinicopathologic, Immunohistochemical, and Molecular Genetic Study of 14 Cases. 61
25651469 2015
18
Proximal-type epithelioid sarcoma-unusual presentation: unilateral vulvar mass. 61
25858929 2015
19
SMARCB1 (INI1)-negative rhabdoid carcinomas of the gastrointestinal tract: clinicopathologic and molecular study of a highly aggressive variant with literature review. 61
24503755 2014
20
Differential microRNA expression profiles between malignant rhabdoid tumor and epithelioid sarcoma: miR193a-5p is suggested to downregulate SMARCB1 mRNA expression. 61
24287458 2014
21
[Epithelioid sarcoma of the orbit]. 61
24337357 2014
22
SMARCB1 expression in epithelioid sarcoma is regulated by miR-206, miR-381, and miR-671-5p on Both mRNA and protein levels. 61
24327545 2014
23
[A case of proximal type epithelioid sarcoma of the perineum]. 61
24322417 2013
24
Vulvar "proximal-type" epithelioid sarcoma: report of a case and review of the literature. 61
23886403 2013
25
Proximal-type epithelioid sarcoma -- case report. 61
23793215 2013
26
Proximal-type Epithelioid Sarcoma of the Head and Neck (HN): A Study with Immunohistochemical and Molecular Analysis of SMARCB1. 61
24308011 2013
27
Proximal-type epithelioid sarcoma of the scrotum: 18F-FDG PET/CT imaging. 61
23242066 2013
28
Proximal-type epithelioid sarcoma of the vulva with INI1 diagnostic utility. 61
21724432 2012
29
Proximal-type epithelioid sarcoma: case report of an unusual presentation. 61
22240247 2012
30
Proximal epithelioid sarcoma of the vulva: a case report and review of current medical literature. 61
22487511 2012
31
Proximal-type Epithelioid Sarcoma Arising in the Inguinal Area. 61
22783522 2012
32
Proximal-type epithelioid sarcoma-a rare soft tissue sarcoma of thigh in a child. 61
23204785 2011
33
p63 immunohistochemical staining is limited in soft tissue tumors. 61
22031315 2011
34
Proximal-type epithelioid sarcoma in pubis. Case report and review of the literature. 61
22169376 2011
35
Intra-articular epithelioid sarcoma showing mixed classic and proximal-type features: report of 2 cases, with immunohistochemical and molecular cytogenetic INI-1 study. 61
21566521 2011
36
An unusual and potentially misleading phenotypic change in a primary gastrointestinal stromal tumour (GIST) under imatinib mesylate therapy. 61
21191613 2011
37
Proximal-type epithelioid sarcoma of the uterine corpus. 61
21255067 2011
38
Proximal-type epithelioid sarcoma of the mons pubis: report of a case. 61
21797131 2011
39
Proximal-type epithelioid sarcoma of the vulva: relationship to malignant extrarenal rhabdoid tumor. 61
20881848 2010
40
Proximal-type epithelioid sarcoma of the maxilla with metastasis to the scalp: Cytologic findings of an unusual malignant tumor. 61
20058307 2010
41
Proximal-type epithelioid sarcoma in the groin presenting as a diagnostic dilemma. 61
19757197 2010
42
Proximal type epithelioid sarcoma arising in the base of the skull: a diagnostic challenge. 61
20360141 2010
43
Epithelioid sarcoma of the parotid gland of a child. 61
19335024 2009
44
[Clinicopathological study of 5 cases of proximal-type epithelioid sarcoma]. 61
19575870 2009
45
Loss of INI1 expression is characteristic of both conventional and proximal-type epithelioid sarcoma. 61
19033866 2009
46
Infrequent SMARCB1/INI1 gene alteration in epithelioid sarcoma: a useful tool in distinguishing epithelioid sarcoma from malignant rhabdoid tumor. 61
18973917 2009
47
A case of proximal-type epithelioid sarcoma which showed positive reactivity to fibroblast growth factor receptor 2-IIIb isotype. 61
18482307 2008
48
Proximal-type epithelioid sarcoma of the scrotum: a difficult diagnosis in an unusual location and review of the literature. 61
18428063 2008
49
Proximal-type epithelioid sarcoma vs. malignant rhabdoid tumor of the vulva: a case report, review of the literature, and an argument for consolidation. 61
17692365 2007
50
[Case of local recurrence of the periurethral proximal-type epithelioid sarcoma successfully treated by repeated regional excision]. 61
18018593 2007

Variations for Central Epithelioid Sarcoma

Expression for Central Epithelioid Sarcoma

Search GEO for disease gene expression data for Central Epithelioid Sarcoma.

Pathways for Central Epithelioid Sarcoma

Pathways related to Central Epithelioid Sarcoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 12.32 VIM KRT7 KRT5 KRT20 KRT18
2
Show member pathways
12.21 KRT7 KRT5 KRT20 KRT18
3 11.99 CD1E CD1D CD1C CD1B CD1A
4 11.85 CD34 CD1D CD1C CD1B CD1A
5
Show member pathways
11.74 VIM KRT7 KRT5 KRT18
6 11.66 CD1E CD1D CD1C CD1B CD1A
7 11.3 MME CD34 CD207 CD1D CD1C CD1A
8 11.28 TNFRSF8 MME CD34 CD1D CD1C
9 11.16 MME CD34 CD1E CD1D CD1C CD1B

GO Terms for Central Epithelioid Sarcoma

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

# Name GO ID Score Top Affiliating Genes
1 integral component of plasma membrane GO:0005887 9.91 PMEL MME CD34 CD1E CD1D CD1C
2 endosome GO:0005768 9.73 PMEL CD1E CD1D CD1C CD1B CD1A
3 endosome membrane GO:0010008 9.62 CD1D CD1C CD1B CD1A
4 keratin filament GO:0045095 9.58 KRT7 KRT5 KRT18
5 intermediate filament GO:0005882 9.55 VIM KRT7 KRT5 KRT20 KRT18
6 lysosome GO:0005764 9.43 TNFAIP3 CD34 CD1E CD1D CD1C CD1B
7 external side of plasma membrane GO:0009897 9.1 CD34 CD1E CD1D CD1C CD1B CD1A

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

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.8 CD1E CD1D CD1C CD1B CD1A
2 keratinization GO:0031424 9.67 KRT7 KRT5 KRT20 KRT18
3 regulation of immune response GO:0050776 9.65 CD34 CD1D CD1C CD1B CD1A
4 cornification GO:0070268 9.62 KRT7 KRT5 KRT20 KRT18
5 positive regulation of T cell mediated cytotoxicity GO:0001916 9.55 CD1E CD1D CD1C CD1B CD1A
6 antigen processing and presentation, exogenous lipid antigen via MHC class Ib GO:0048007 9.35 CD1E CD1D CD1C CD1B CD1A
7 antigen processing and presentation, endogenous lipid antigen via MHC class Ib GO:0048006 9.02 CD1E CD1D CD1C CD1B CD1A

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

# Name GO ID Score Top Affiliating Genes
1 lipopeptide binding GO:0071723 9.55 CD1E CD1D CD1C CD1B CD1A
2 structural constituent of cytoskeleton GO:0005200 9.5 VIM KRT5 KRT20
3 scaffold protein binding GO:0097110 9.43 VIM KRT5 KRT18
4 exogenous lipid antigen binding GO:0030884 9.35 CD1E CD1D CD1C CD1B CD1A
5 endogenous lipid antigen binding GO:0030883 9.02 CD1E CD1D CD1C CD1B CD1A

Sources for Central Epithelioid Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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