MCID: KDN007
MIFTS: 37

Kidney Clear Cell Sarcoma

Categories: Nephrological diseases, Rare diseases, Cancer diseases

Aliases & Classifications for Kidney Clear Cell Sarcoma

MalaCards integrated aliases for Kidney Clear Cell Sarcoma:

Name: Kidney Clear Cell Sarcoma 12 15
Clear Cell Sarcoma of Kidney 12 59 73
Childhood Kidney Clear Cell Sarcoma 12
Pediatric Renal Clear Cell Sarcoma 12
Renal Clear Cell Sarcoma 12
Ccsk 59

Characteristics:

Orphanet epidemiological data:

59
clear cell sarcoma of kidney
Inheritance: Not applicable; Age of onset: Adolescent,Adult,Childhood,Infancy;

Classifications:

Orphanet: 59  
Rare renal diseases


External Ids:

Disease Ontology 12 DOID:4880
NCIt 50 C4264
SNOMED-CT 68 189813000 24007003
Orphanet 59 ORPHA457246
ICD10 via Orphanet 34 C64
UMLS 73 C0334488

Summaries for Kidney Clear Cell Sarcoma

Disease Ontology : 12 A kidney sarcoma that has material basis in clear cells that are characterized as cells that look clear when viewed under a microscope.

MalaCards based summary : Kidney Clear Cell Sarcoma, also known as clear cell sarcoma of kidney, is related to clear cell sarcoma and kidney sarcoma. An important gene associated with Kidney Clear Cell Sarcoma is NUTM2E (NUT Family Member 2E), and among its related pathways/superpathways are Neural Stem Cell Differentiation Pathways and Lineage-specific Markers and Regulation of Wnt-mediated beta catenin signaling and target gene transcription. The drugs Etoposide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include kidney, lung and bone.

Wikipedia : 76 Clear cell sarcoma of the kidney (CCSK) is an extremely rare type of kidney cancer comprising 3% of all... more...

Related Diseases for Kidney Clear Cell Sarcoma

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

# Related Disease Score Top Affiliating Genes
1 clear cell sarcoma 30.1 BCOR WT1 YWHAE
2 kidney sarcoma 29.0 TH WT1
3 myxoid leiomyosarcoma 10.1 BCOR YWHAE
4 endometrial stromal nodule 10.0 BCOR YWHAE
5 sarcoma 9.8
6 endometrial stromal sarcoma 9.8 BCOR NUTM2B YWHAE
7 hydrocephalus due to congenital stenosis of aqueduct of sylvius 9.4 NES TH
8 leukemia, acute myeloid 9.3 BCOR TERT WT1
9 rhabdomyosarcoma 2 9.0 TH WT1

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



Diseases related to Kidney Clear Cell Sarcoma

Symptoms & Phenotypes for Kidney Clear Cell Sarcoma

Drugs & Therapeutics for Kidney Clear Cell Sarcoma

Drugs for Kidney Clear Cell Sarcoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 85)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Etoposide Approved Phase 3,Phase 2,Phase 1 33419-42-0 36462
2
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
3
Carboplatin Approved Phase 3,Phase 2,Phase 1 41575-94-4 10339178 498142 38904
4
Vincristine Approved, Investigational Phase 3,Phase 2 2068-78-2, 57-22-7 5978
5
Cyclophosphamide Approved, Investigational Phase 3,Phase 2 50-18-0, 6055-19-2 2907
6
Dactinomycin Approved, Investigational Phase 3,Phase 2 50-76-0 2019 457193
7
Lenograstim Approved, Investigational Phase 3,Phase 2 135968-09-1
8
Sulfamethoxazole Approved Phase 3 723-46-6 5329
9
Mechlorethamine Approved, Investigational Phase 3,Phase 2,Phase 1 51-75-2 4033
10
Ifosfamide Approved Phase 3,Phase 2,Phase 1 3778-73-2 3690
11
Trimethoprim Approved, Vet_approved Phase 3 738-70-5 5578
12
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
13
Cyproheptadine Approved Phase 3 129-03-3 2913
14
Histamine Approved, Investigational Phase 3 75614-87-8, 51-45-6 774
15
Doxil Approved June 1999 Phase 3,Phase 2 31703
16 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
17 Alkylating Agents Phase 3,Phase 2,Phase 1
18 Topoisomerase Inhibitors Phase 3,Phase 2,Phase 1
19 Nucleic Acid Synthesis Inhibitors Phase 3,Phase 2
20 Anti-Bacterial Agents Phase 3,Phase 2,Phase 1
21 Antibiotics, Antitubercular Phase 3,Phase 2
22 Etoposide phosphate Phase 3,Phase 2,Phase 1
23 Immunosuppressive Agents Phase 3,Phase 2,Phase 1
24 Anti-Infective Agents Phase 3,Phase 2,Phase 1
25 Antirheumatic Agents Phase 3,Phase 2
26 Antimitotic Agents Phase 3,Phase 2,Phase 1,Not Applicable
27 Antineoplastic Agents, Alkylating Phase 3,Phase 2,Phase 1
28 Antineoplastic Agents, Phytogenic Phase 3,Phase 2,Phase 1,Not Applicable
29
Isophosphamide mustard Phase 3,Phase 2,Phase 1 0
30 Analgesics Phase 3
31 Central Nervous System Depressants Phase 3
32 Adjuvants, Anesthesia Phase 3
33 Adjuvants, Immunologic Phase 3,Phase 2
34 Narcotics Phase 3
35 Analgesics, Opioid Phase 3
36 Anesthetics Phase 3
37 Anesthetics, General Phase 3
38 Anesthetics, Intravenous Phase 3
39 Peripheral Nervous System Agents Phase 3
40 Liver Extracts Phase 3,Phase 1,Phase 2,Not Applicable
41
Histamine Phosphate Phase 3 51-74-1 65513
42 Serotonin Agents Phase 3
43 Serotonin Antagonists Phase 3
44 Gastrointestinal Agents Phase 3
45 Neurotransmitter Agents Phase 3
46 Histamine Antagonists Phase 3
47 Histamine H1 Antagonists Phase 3
48 Dermatologic Agents Phase 3
49 Anti-Allergic Agents Phase 3
50 Antipruritics Phase 3

Interventional clinical trials:

(show all 24)
# Name Status NCT ID Phase Drugs
1 Chemotherapy With or Without Surgery, Radiation Therapy, or Stem Cell Transplantation in Treating Young Patients With Kidney Tumors Completed NCT00002610 Phase 3 carboplatin;cyclophosphamide;doxorubicin hydrochloride;etoposide;vincristine sulfate
2 Combination Chemotherapy Alone or With Radiation Therapy in Treating Children With Kidney Cancer Completed NCT00002611 Phase 3 cyclophosphamide;doxorubicin hydrochloride;etoposide;vincristine sulfate
3 Fentanyl Sublingual Spray in Treating Patients With Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
4 Surgery With or Without Combination Chemotherapy in Treating Patients With Lung Metastases From Soft Tissue Sarcoma Completed NCT00002764 Phase 3 doxorubicin hydrochloride;ifosfamide
5 Surgery With or Without Chemotherapy in Treating Patients With Soft Tissue Sarcoma Completed NCT00002641 Phase 3 doxorubicin hydrochloride;ifosfamide;isolated perfusion
6 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
7 Chemotherapy Followed by Surgery and Radiation Therapy With or Without Stem Cell Transplant in Treating Patients With Relapsed or Refractory Wilms' Tumor or Clear Cell Sarcoma of the Kidney Unknown status NCT00025103 Phase 2 carboplatin;cyclophosphamide;doxorubicin hydrochloride;etoposide;melphalan;vincristine sulfate
8 Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors Completed NCT00335556 Phase 2 Doxorubicin Hydrochloride;Irinotecan Hydrochloride;Cyclophosphamide;Etoposide;Carboplatin;Vincristine Sulfate
9 Therapeutic Angiotensin-(1-7) in Treating Patients With Metastatic Sarcoma That Cannot Be Removed By Surgery Completed NCT01553539 Phase 2 therapeutic angiotensin-(1-7)
10 Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01154452 Phase 1, Phase 2 Gamma-Secretase Inhibitor RO4929097;Vismodegib
11 Chemotherapy in Treating Patients With Solid Tumors Completed NCT00003103 Phase 1, Phase 2 docetaxel
12 High-dose ICE With Amifostine Completed NCT00003657 Phase 2 Amifostine;Carboplatin;Etoposide;Ifosfamide
13 Sunitinib Malate With or Without Gemcitabine Hydrochloride in Treating Patients With Advanced Kidney Cancer That Cannot Be Removed By Surgery Active, not recruiting NCT01164228 Phase 2 gemcitabine hydrochloride;sunitinib malate
14 Dasatinib, Ifosfamide, Carboplatin, and Etoposide in Treating Young Patients With Metastatic or Recurrent Malignant Solid Tumors Active, not recruiting NCT00788125 Phase 1, Phase 2 carboplatin;dasatinib;etoposide phosphate;ifosfamide
15 Dalteparin and Sunitinib Malate as First-Line Therapy in Treating Patients With Kidney Cancer That is Metastatic or Cannot Be Removed by Surgery Completed NCT01061411 Phase 1 Dalteparin;Sunitinib Malate
16 Thalidomide and Docetaxel in Treating Patients With Advanced Cancer Completed NCT00049296 Phase 1 docetaxel;thalidomide
17 Study of Information Received About Fertility and Infertility in Patients Who Have Finished Treatment for Cancer Unknown status NCT01295463
18 Study of Genes and Environment in Patients With Cancer in East Anglia, Trent, or West Midlands Regions of the United Kingdom Unknown status NCT00757614
19 Biomarkers in Tissue Samples From Patients With High-Risk Wilms Tumor Completed NCT01118078
20 E-MOSAIC Electronic Tool to Monitor Symptoms Completed NCT00477919
21 Early Intervention vs. Standard Palliative Care in Improving End-of-Life Care in Advanced Cancer Patients Completed NCT00253383 Not Applicable
22 Study of Kidney Tumors in Younger Patients Recruiting NCT00898365
23 Studying Biomarkers in Samples From Younger Patients With Kidney Cancer Withdrawn NCT01642095
24 Paclitaxel Albumin-Stabilized Nanoparticle Formulation in Treating Patients With Advanced or Refractory Solid Tumors Withdrawn NCT00499291 Not Applicable paclitaxel albumin-stabilized nanoparticle formulation

Search NIH Clinical Center for Kidney Clear Cell Sarcoma

Genetic Tests for Kidney Clear Cell Sarcoma

Anatomical Context for Kidney Clear Cell Sarcoma

MalaCards organs/tissues related to Kidney Clear Cell Sarcoma:

41
Kidney, Lung, Bone, Brain, Liver

Publications for Kidney Clear Cell Sarcoma

Articles related to Kidney Clear Cell Sarcoma:

(show all 24)
# Title Authors Year
1
Delayed Metastasis of Clear Cell Sarcoma of Kidney to Bladder After 7 Disease-Free Years. ( 29509095 )
2018
2
FNAC aided diagnosis of clear cell sarcoma of kidney: Report of two cases in infants. ( 28421721 )
2017
3
Clear cell sarcoma of kidney involving a horseshoe kidney and harboring EGFR internal tandem duplication. ( 28440018 )
2017
4
Concurrent occurrence of neuroblastoma and clear cell sarcoma of kidney in an infant: Is it a random event? ( 28195107 )
2017
5
Primary Renal Sarcomas With BCOR-CCNB3 Gene Fusion: A Report of 2 Cases Showing Histologic Overlap With Clear Cell Sarcoma of Kidney, Suggesting Further Link Between BCOR-related Sarcomas of the Kidney and Soft Tissues. ( 28817404 )
2017
6
Dual tumor - Clear cell sarcoma of kidney with differentiating neuroblastoma: A rare case with review of literature. ( 28195106 )
2017
7
Recurrent BCOR Internal Tandem Duplication and YWHAE-NUTM2B Fusions in Soft Tissue Undifferentiated Round Cell Sarcoma of Infancy: Overlapping Genetic Features With Clear Cell Sarcoma of Kidney. ( 26945340 )
2016
8
Mutually exclusive BCOR internal tandem duplications and YWHAE-NUTM2 fusions in clear cell sarcoma of kidney: not the full story. ( 27000436 )
2016
9
Clear cell sarcoma of kidney in a neonate. ( 26023506 )
2014
10
Clear cell sarcoma of kidney: morphoproteomic analysis reveals genomic correlates and therapeutic options. ( 22256788 )
2013
11
Outcomes of patients with revised stage I clear cell sarcoma of kidney treated in National Wilms Tumor Studies 1-5. ( 22658515 )
2013
12
Characterization of the chromosomal translocation t(10;17)(q22;p13) in clear cell sarcoma of kidney. ( 22294382 )
2012
13
Clear cell sarcoma of kidney: A rare entity. ( 22557783 )
2011
14
Translocation (10;17)(q22;p13): a recurring translocation in clear cell sarcoma of kidney. ( 15474157 )
2004
15
Clear cell sarcoma of kidney in an adolescent and in young adults: a report of four cases with ultrastructural, immunohistochemical, and DNA flow cytometric analysis. ( 10584698 )
1999
16
Veno-occlusive disease-like hepatotoxicity in two children receiving chemotherapy for Wilms' tumor and clear cell sarcoma of kidney. ( 9509511 )
1998
17
Clear cell sarcoma of kidney with tumor extension into the right atrium. ( 9788798 )
1998
18
Clear cell sarcoma of kidney in an adult. ( 8149160 )
1993
19
Clear cell sarcoma of kidney. Two cases in adults. ( 8453549 )
1993
20
Clear cell sarcoma of kidney: a clinicopathological study of eight cases from Malaysia. ( 1328018 )
1992
21
Clear cell sarcoma of kidney: report of the first Malaysian case. ( 1374551 )
1992
22
Primitive pelvic sarcoma resembling clear cell sarcoma of kidney. ( 1755102 )
1991
23
Clear cell sarcoma of kidney (CCSK)--a clinicopathological study of 4 cases. ( 2591997 )
1989
24
Case 7. Clear cell sarcoma of kidney. ( 2748484 )
1989

Variations for Kidney Clear Cell Sarcoma

Cosmic variations for Kidney Clear Cell Sarcoma:

9
(show all 30)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM4169595 DICER1 soft tissue,kidney,sarcoma,NS c.5425G>A p.G1809R 14:95091305-95091305 15
2 COSM6979022 SRC soft tissue,lung,sarcoma,NS c.205T>G p.S69A 20:37384358-37384358 10
3 COSM389868 RAC1 soft tissue,lung,sarcoma,NS c.476C>T p.A159V 7:6402343-6402343 10
4 COSM6979014 PTPRD soft tissue,lung,sarcoma,NS c.4160C>T p.A1387V 9:8404587-8404587 10
5 COSM6959031 PTCH1 soft tissue,lung,sarcoma,NS c.2921T>G p.F974C 9:95458260-95458260 10
6 COSM6979006 PBRM1 soft tissue,lung,sarcoma,NS c.2834G>A p.R945H 3:52589201-52589201 10
7 COSM6979021 PAK5 soft tissue,lung,sarcoma,NS c.41C>A p.P14Q 20:9644288-9644288 10
8 COSM520 KRAS soft tissue,lung,sarcoma,NS c.35G>T p.G12V 12:25245350-25245350 10
9 COSM516 KRAS soft tissue,lung,sarcoma,NS c.34G>T p.G12C 12:25245351-25245351 10
10 COSM6979011 HGF soft tissue,lung,sarcoma,NS c.1711G>T p.V571L 7:81706333-81706333 10
11 COSM1732355 H3F3A soft tissue,bone,sarcoma,NS c.103G>T p.G35W 1:226064454-226064454 10
12 COSM6959034 GPS2 soft tissue,lung,sarcoma,NS c.454C>T p.Q152* 17:7313932-7313932 10
13 COSM3908208 GNAQ soft tissue,bone,sarcoma,NS c.442C>T p.R148* 9:77815650-77815650 10
14 COSM6979008 FOXP1 soft tissue,lung,sarcoma,NS c.74A>G p.H25R 3:71198308-71198308 10
15 COSM3908209 soft tissue,bone,sarcoma,NS c.355C>T p.R119* 9:77815650-77815650 10
16 COSM6959030 soft tissue,lung,sarcoma,NS c.2921T>G p.F974C 9:95458260-95458260 10
17 COSM6979007 soft tissue,lung,sarcoma,NS c.2834G>A p.R945H 3:52589201-52589201 10
18 COSM6979005 soft tissue,lung,sarcoma,NS c.2738G>A p.R913H 3:52589201-52589201 10
19 COSM6979012 soft tissue,lung,sarcoma,NS c.4160C>T p.A1387V 9:8404587-8404587 10
20 COSM6979020 soft tissue,lung,sarcoma,NS c.1831+1G>T p.? 19:45353082-45353082 10
21 COSM1140136 soft tissue,lung,sarcoma,NS c.34G>T p.G12C 12:25245351-25245351 10
22 COSM6979017 soft tissue,lung,sarcoma,NS c.2573C>T p.A858V 9:8404587-8404587 10
23 COSM6979010 soft tissue,lung,sarcoma,NS c.1036G>T p.V346L 3:179203766-179203766 10
24 COSM6979009 soft tissue,lung,sarcoma,NS c.74A>G p.H25R 3:71198308-71198308 10
25 COSM1154840 soft tissue,lung,sarcoma,NS c.533C>T p.A178V 7:6402343-6402343 10
26 COSM6959033 soft tissue,lung,sarcoma,NS c.2918T>G p.F973C 9:95458260-95458260 10
27 COSM6979016 soft tissue,lung,sarcoma,NS c.2930C>T p.A977V 9:8404587-8404587 10
28 COSM6979013 soft tissue,lung,sarcoma,NS c.2942C>T p.A981V 9:8404587-8404587 10
29 COSM6959032 soft tissue,lung,sarcoma,NS c.2918T>G p.F973C 9:95458260-95458260 10
30 COSM6979015 soft tissue,lung,sarcoma,NS c.2939C>T p.A980V 9:8404587-8404587 10

Expression for Kidney Clear Cell Sarcoma

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

Pathways for Kidney Clear Cell Sarcoma

GO Terms for Kidney Clear Cell Sarcoma

Biological processes related to Kidney Clear Cell Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cerebral cortex development GO:0021987 8.96 TH YWHAE
2 heart development GO:0007507 8.8 BCOR TH WT1

Sources for Kidney Clear Cell 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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