MCID: CLR017
MIFTS: 48

Clear Cell Sarcoma malady

Cancer category

Summaries for Clear Cell Sarcoma

Sources:
8Disease Ontology, 64Wikipedia, 33MalaCards
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Disease Ontology:8 A sarcoma that is characterized by solid nests and fascicles of tumor cells with clear cytoplasm and prominent nucleoli. it presents as a slow growing mass that especially affects tendons and aponeuroses and it is deeply situated.

MalaCards: Clear Cell Sarcoma, also known as melanoma, malignant, of soft parts, is related to wilms tumor and spindle cell sarcoma. An important gene associated with Clear Cell Sarcoma is ATF1 (activating transcription factor 1), and among its related pathways are RSK activation and Striated Muscle Contraction. The compounds glycogen and creatinine have been mentioned in the context of this disorder. Affiliated tissues include lymph node, brain and small intestine, and related mouse phenotypes are muscle and normal.

Wikipedia:64 Clear-cell sarcoma (formerly known as malignant melanoma of the soft parts) is a rare form of cancer... more...

Aliases & Classifications for Clear Cell Sarcoma

Sources:
8Disease Ontology, 10DISEASES, 45Novoseek, 61UMLS, 57SNOMED-CT, 40NCIt, 35MeSH
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer


Aliases & Descriptions:

clear cell sarcoma 8 10
melanoma, malignant, of soft parts 8
adult soft part clear cell sarcoma 8
malignant melanoma of soft tissues 8
clear cell sarcoma of soft parts 8
sarcoma, clear cell 61
sarcoma clear cell 45


External Ids:

Disease Ontology8 DOID:4233
NCIt40 C3745
MeSH35 D018227

Related Diseases for Clear Cell Sarcoma

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the Clear Cell Sarcoma family:

small cell sarcoma

Diseases related to Clear Cell Sarcoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 138)
idRelated DiseaseScoreTop Affiliating Genes
1wilms tumor30.9ENO2, ACTC1, DES, EWSR1, H19, VIM
2spindle cell sarcoma30.5VIM, S100B, DES, ACTC1
3adenoma30.5S100A1, H19, CREB1, ACTC1, ENO2
4synovial sarcoma30.4VIM, S100B, DES
5alveolar soft part sarcoma30.2TFE3
6myxoid liposarcoma30.1ATF1, DDIT3, EWSR1
7paraganglioma30.1ENO2, S100A1, S100B
8pleomorphic adenoma30.1VIM, S100B, S100A1, DES, ACTC1
9microphthalmia30.1S100B, TFE3, CREB1, MLANA, ACTC1
10kidney clear cell sarcoma11.1
11soft tissue sarcoma10.8
12small cell sarcoma10.5
13clear cell adenoma10.4
14mesoblastic nephroma10.4
15brain sarcoma10.3
16sarcomatosis10.3
17cutaneous malignant melanoma10.3
18melanoma of soft part10.3
19amelanotic melanoma10.1
20familial adenomatous polyposis10.1
21pleomorphic liposarcoma10.1
22epithelioid cell synovial sarcoma10.1
23penis sarcoma10.1
24metanephric adenoma10.1
25acral lentiginous melanoma10.1
26pediatric lymphoma10.1
27clear cell chondrosarcoma10.1
28arthritis10.1
29skin sarcoma10.1
30kid syndrome10.1
31spitz nevus10.1
32nephroblastoma10.0EWSR1
33infantile myofibromatosis10.0DES
34epulis10.0ENO2
35histiocytosis10.0S100B
36leydig cell tumor10.0CREB1
37fasciitis10.0ACTC1
38neuroblastoma10.0ENO2
39perivascular epithelioid cell tumor10.0TFE3, MLANA
40rhabdoid cancer10.0ENO2
41neurilemmoma10.0ENO2
42subependymal giant cell astrocytoma10.0ENO2
43syringoma10.0S100A1, S100B
44bladder carcinoma10.0H19
45lymphangioleiomyomatosis10.0MLANA, DES
46fibrous meningioma10.0S100B
47cerebral primitive neuroectodermal tumor10.0EWSR1, ENO2
48hemangioblastoma10.0ENO2, S100B
49hemangioma10.0ACTC1
50oxyphilic adenoma10.0S100A1, TFE3

Graphical network of the top 20 diseases related to Clear Cell Sarcoma:



Diseases related to clear cell sarcoma

Clinical Features for Clear Cell Sarcoma

Drugs & Therapeutics for Clear Cell Sarcoma

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Clear Cell Sarcoma

Drug clinical trials:

Search ClinicalTrials for Clear Cell Sarcoma

Search NIH Clinical Center for Clear Cell Sarcoma

Search CenterWatch for Clear Cell Sarcoma

Genetic Tests for Clear Cell Sarcoma

Anatomical Context for Clear Cell Sarcoma

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Clear Cell Sarcoma:

33
Lymph node, Brain, Small intestine, Colon, Kidney, Lung, Skin, Ovary, T cells, Tongue

Animal Models for Clear Cell Sarcoma or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Clear Cell Sarcoma:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000536910.0ERBB3, ACTC1, CREB1, H19
2MP:00028739.1ERBB3, S100B, S100A1, H19, EWSR1, TFE3

Publications for Clear Cell Sarcoma

Sources:
51PubMed
See all sources

Articles related to Clear Cell Sarcoma:

(show top 50)    (show all 374)
idTitleAuthorsYear
1
Compound clear cell sarcoma misdiagnosed as a Spitz nevus. (23980901)
2013
2
Clear cell sarcoma of the tongue. (24117610)
2013
3
Incidental capture of rarely diagnosed pediatric tumor: an infant boy with clear cell sarcoma of the kidney. (23972342)
2013
4
Clear cell sarcoma of kidney: morphoproteomic analysis reveals genomic correlates and therapeutic options. (22256788)
2013
5
Two cases of clear cell sarcoma with different clinical and genetic features: cutaneous type with BRAF mutation and subcutaneous type with KIT mutation. (23796270)
2013
6
Deep intronic point mutations of the KIT gene in a female patient with cutaneous clear cell sarcoma and her family. (22559980)
2012
7
Sarcomatoid conversion of clear cell renal cell carcinoma in relation to epithelial-to-mesenchymal transition. (21992819)
2012
8
Clear cell sarcoma of kidney: A rare entity. (22557783)
2011
9
Clear cell sarcoma of soft tissue: diagnostic dilemma in cytologic appearance. (20307007)
2010
10
Cutaneous clear cell sarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 12 cases emphasizing its distinction from dermal melanoma. (20087159)
2010
11
Clear cell sarcoma of soft tissue metastatic to the ovaries: a heretofore unreported occurrence. (19620941)
2009
12
Clear cell sarcoma of the stomach. (19350639)
2009
13
Detection and characterization of EWSR1/ATF1 and EWSR1/CREB1 chimeric transcripts in clear cell sarcoma (melanoma of soft parts). (19561568)
2009
14
Adult clear cell sarcoma of the kidney: a case report. (16584570)
2006
15
Osteoclast-rich tumor of the gastrointestinal tract with features resembling those of clear cell sarcoma of soft parts. (16220298)
2006
16
Is clear cell sarcoma a malignant form of psammomatous melanotic schwannoma? Case report. (17341045)
2006
17
Clear cell sarcoma of tendons and aponeuroses: a historical perspective and tribute to the man behind the entity. (17075294)
2006
18
Clear cell sarcoma (malignant melanoma of soft parts) and sentinel lymph node biopsy. (15701594)
2005
19
MR appearance of clear cell sarcoma of tendons and aponeuroses (malignant melanoma of soft parts): radiologic-pathologic correlation. (16132975)
2005
20
Alterations of the p16INK4a/p14ARF pathway in clear cell sarcoma. (15298727)
2004
21
Cytogenetic comparison between clear cell sarcoma and a case of acral clear cell melanoma. (16285301)
2003
22
Clear cell sarcoma shows immunoreactivity for microphthalmia transcription factor: further evidence for melanocytic differentiation. (11211309)
2001
23
Poorly differentiated endometrial sarcoma with clear cell features. (11890573)
2001
24
Clear cell sarcoma of soft parts. A case report and review of the literature. (11398297)
2001
25
Clear cell sarcoma: the Roswell Park experience. (11344475)
2001
26
MR imaging of clear cell sarcoma (malignant melanoma of the soft parts): a multicenter correlative MRI-pathology study of 21 cases and literature review. (10855466)
2000
27
Soft tissue Ewing sarcoma--peripheral primitive neuroectodermal tumor with atypical clear cell pattern shows a new type of EWS-FEV fusion transcript. (10976720)
2000
28
Tyrosinase gene expression in clear cell sarcoma indicates a melanocytic origin: insight from the first reported canine case. (10598869)
1999
29
Clear cell sarcoma of soft tissues in children and young adults: the St. Jude Children's Research Hospital experience. (10599094)
1999
30
Clear cell sarcoma kidney: clinical features and outcome. (10100278)
1999
31
Clear cell sarcoma of the kidney: an unusual presentation and review of the literature. (9544171)
1998
32
Febrile arthritis as the first manifestation of clear cell sarcoma of the tendons and aponeuroses. (9809366)
1998
33
Clear cell sarcoma: an extremely rare cause of pleural disease. (9426110)
1997
34
Newly established MST-1 tumour cell line and tumour-infiltrating lymphocyte culture from a patient with soft tissue melanoma (clear cell sarcoma) and their potential applications to patient immunotherapy. (8664053)
1996
35
Clear cell sarcoma of the kidney in an adult. (8522434)
1995
36
Aberrations of chromosome 22 and polysomy of chromosome 8 as non-random changes in clear cell sarcoma. (8143273)
1994
37
Treatment of children with clear-cell sarcoma of the kidney: a report from the National Wilms' Tumor Study Group. (7931484)
1994
38
Clear cell sarcoma (malignant melanoma of soft parts). Presentation of two additional cases. (8491327)
1993
39
Clear cell sarcoma of tendons and aponeuroses and malignant blue nevus arising in prepubescent children. Report of two cases and review of the literature. (8494114)
1993
40
Clear cell sarcoma with t(12;22) (q13-14;q12). (1797088)
1991
41
Round cell sarcoma after clear cell sarcoma remission. (1835638)
1991
42
Cytogenetic findings in clear cell sarcoma of tendons and aponeuroses. Malignant melanoma of soft parts. (2009504)
1991
43
Chromosomal abnormalities in clear cell sarcoma. Implications for histogenesis. (2294702)
1990
44
Adult clear cell sarcoma of the kidney. Case report and review of literature. (2512069)
1989
45
Sarcomatous Wilms' tumour with clear cells and hyalinization. A study of 38 tumours in children from the SIOP nephroblastoma file. (2448218)
1987
46
Implant clear cell sarcoma (malignant melanoma of soft parts). (3612000)
1987
47
Case report 423: Clear-cell sarcoma plantar aspect of right foot. (3616672)
1987
48
Clear cell sarcoma of the kidney: a clinicopathologic study of 21 patients with long-term follow-up evaluation. (4065884)
1985
49
Clear cell sarcoma. A clinicopathologic study of 27 cases. (6616410)
1983
50
Clear-cell sarcoma of palmar aponeurosis and tendons. (977056)
1976

Genetic Variations for Clear Cell Sarcoma

Expression for genes affiliated with Clear Cell Sarcoma

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Clear Cell Sarcoma

Search GEO for disease gene expression data for Clear Cell Sarcoma.

Pathways for genes affiliated with Clear Cell Sarcoma

Sources:
54Reactome, 38NCBI BioSystems Database, 30KEGG, 52QIAGEN
See all sources

Pathways related to Clear Cell Sarcoma according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.2ATF1, CREB1
2
Hide members
10.1VIM, DES, ACTC1
310.1ATF1, DDIT3, CREB1
49.9ATF1, DDIT3, TFE3, EWSR1
5
Hide members
9.7ERBB3, DES, ATF1, DDIT3, CREB1, VIM

Compounds for genes affiliated with Clear Cell Sarcoma

Sources:
45Novoseek, 24HMDB, 50PharmGKB, 2BitterDB, 11DrugBank, 29IUPHAR, 60Tocris Bioscience
See all sources

Compounds related to Clear Cell Sarcoma according to GeneCards/GeneDecks:

(show top 50)    (show all 53)
idCompoundScoreTop Affiliating Genes
1glycogen45 2411.6ACTC1, CREB1
2creatinine4510.6ACTC1
3askin4510.5EWSR1, ENO2
42-methoxyethanol4510.5ACTC1, S100A1, VIM
5orcein4510.4VIM, ACTC1, DES, S100B
6edss4510.4S100B, ENO2
7reticulin4510.4DES, ACTC1, S100B, VIM
8eosin4510.4VIM, S100B
9actomyosin4510.4ACTC1, VIM, S100A1
10azathioprine45 50 2 1113.3CREB1, H19, VIM, DES
11toluidine4510.3ENO2, DES, S100A1, VIM
12acetylcholine45 50 29 11 2414.3ERBB3, S100A1, ENO2, CREB1
13glucose4510.3ERBB3, ACTC1, DDIT3, TFE3
14mucicarmine4510.3S100B, VIM, ACTC1, DES, S100A1
15dbc-amp4510.3VIM, S100B, ENO2, CREB1
16b 7234510.3VIM, S100B, S100A1, DES, ACTC1
17biotin45 11 2412.3S100A1, DES, ACTC1, VIM
18s 1004510.2VIM, S100B, DES, ACTC1, ENO2
19glutamine4510.2ENO2, DDIT3, CREB1, EWSR1
20agar4510.2CREB1, ERBB3, H19, VIM
21formaldehyde45 2411.2VIM, S100B, DES, ENO2, ACTC1
22acth4510.2ENO2, S100B, CREB1, H19
23ganglioside4510.1ENO2, VIM, DES
24bromodeoxyuridine4510.1S100B, DDIT3, ENO2, VIM, DES
25norepinephrine45 11 2412.1CREB1, S100B, ATF1, ENO2, DES
26gaba4510.1CREB1, S100B, ENO2, VIM
27cyclic amp45 2411.1CREB1, ATF1, EWSR1, S100B, DES, VIM
28etoposide45 50 60 1113.1VIM, ENO2, S100B, DDIT3, CREB1
29forskolin45 50 1112.1ATF1, CREB1, VIM, S100B, ENO2, ERBB3
30adenylate4510.0VIM, DES, CREB1, ENO2, ATF1, S100B
31steroid4510.0ERBB3, DES, ATF1, CREB1, H19, ACTC1
32lactate4510.0ENO2, DES, MLANA, CREB1, S100B, VIM
33zinc45 2411.0S100A1, CREB1, MFI2, EWSR1, H19, S100B
3412-o-tetradecanoylphorbol 13-acetate4510.0VIM, ATF1, DES, CREB1
35heparin45 29 11 2413.0ERBB3, ENO2, DES, S100B, VIM
36hematoxylin459.9MLANA, DES, ACTC1, VIM, S100A1, S100B
37methionine459.9DES, S100B, VIM, DDIT3
38leucine459.9VIM, ENO2, ATF1, DDIT3, CREB1, TFE3
39progesterone45 60 29 11 2413.8VIM, S100A1, S100B, ATF1, DES, ACTC1
40sb 20358045 6010.8ATF1, DDIT3, CREB1, VIM
41testosterone45 60 11 2412.8CREB1, ERBB3, DES, S100B, VIM, ACTC1
42iron45 2410.8DES, ACTC1, DDIT3, S100A1, S100B, VIM
43vegf459.8DDIT3, DES, VIM, ENO2, ERBB3, ACTC1
44atp45 2910.7DDIT3, CREB1, S100B, ENO2, S100A11, VIM
45silver459.7S100A1, ENO2, DES
46oxygen45 2410.7DES, S100B, ENO2, ACTC1, MFI2, DDIT3
47paraffin459.6H19, VIM, S100B, S100A1, EWSR1, MLANA
48retinoic acid45 2410.5CREB1, EWSR1, S100B, S100A1, H19, DDIT3
49tyrosine459.5ENO2, ERBB3, ACTC1, DES, DDIT3, CREB1
50calcium45 50 11 2412.3S100A11, DES, ENO2, ERBB3, ATF1, DDIT3

GO Terms for genes affiliated with Clear Cell Sarcoma

Sources:
16Gene Ontology
See all sources

Biological processes related to Clear Cell Sarcoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1muscle filament slidingGO:03004910.1ACTC1, DES, VIM

Molecular functions related to Clear Cell Sarcoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1S100 protein bindingGO:0445489.9S100A11, S100A1, S100B
2identical protein bindingGO:0428029.4VIM, S100B, S100A1, EWSR1, DES, ERBB3
3protein bindingGO:0055158.7S100B, S100A1, S100A11, EWSR1, MFI2, CREB1

Products for genes affiliated with Clear Cell Sarcoma

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Sources for Clear Cell Sarcoma

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet