MCID: KDN019
MIFTS: 35

Kidney Sarcoma

Categories: Cancer diseases, Nephrological diseases

Aliases & Classifications for Kidney Sarcoma

MalaCards integrated aliases for Kidney Sarcoma:

Name: Kidney Sarcoma 12 15
Sarcoma of Kidney 12 73
Renal Sarcoma 12 29

Classifications:



External Ids:

Disease Ontology 12 DOID:4242
NCIt 50 C4525
UMLS 73 C0346251

Summaries for Kidney Sarcoma

Disease Ontology : 12 A kidney cancer that is located in the kidney's connective tissue.

MalaCards based summary : Kidney Sarcoma, also known as sarcoma of kidney, is related to kidney clear cell sarcoma and clear cell sarcoma. An important gene associated with Kidney Sarcoma is BCOR (BCL6 Corepressor), and among its related pathways/superpathways are Regulation of Telomerase and Signaling events mediated by HDAC Class II. The drugs Dactinomycin and Carboplatin have been mentioned in the context of this disorder. Affiliated tissues include kidney, t cells and lung, and related phenotypes are Decreased shRNA abundance (Z-score < -2) and Decreased shRNA abundance (Z-score < -2)

Related Diseases for Kidney Sarcoma

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

(show all 45)
# Related Disease Score Top Affiliating Genes
1 kidney clear cell sarcoma 30.8 BCOR MB WT1 YWHAE
2 clear cell sarcoma 29.4 BCOR WT1 YWHAE
3 kidney osteogenic sarcoma 11.0
4 kidney leiomyosarcoma 10.9
5 myxoid leiomyosarcoma 10.1 BCOR YWHAE
6 endometrial stromal nodule 10.1 BCOR YWHAE
7 endometrial stromal tumor 10.0 BCOR YWHAE
8 sarcoma, synovial 10.0
9 sarcoma 10.0
10 pleuropulmonary blastoma 10.0 DICER1 MB
11 leiomyosarcoma 9.9 MB WT1
12 kidney rhabdoid cancer 9.9 MB WT1
13 endometrial stromal sarcoma 9.9 BCOR MB YWHAE
14 adult fibrosarcoma 9.9 MB WT1
15 testicular granulosa cell tumor 9.9 DICER1 WT1
16 juvenile type testicular granulosa cell tumor 9.9 DICER1 WT1
17 male reproductive organ benign neoplasm 9.9 DICER1 WT1
18 cystic nephroma 9.9 DICER1 WT1
19 kidney benign neoplasm 9.9 DICER1 WT1
20 malignant mesenchymoma 9.8 MB WT1
21 neuroblastoma 9.8
22 wilms tumor 6 9.8
23 sertoli-leydig cell tumor 9.8 DICER1 WT1
24 uterine sarcoma 9.8 MB WT1
25 embryonal rhabdomyosarcoma 9.8 DICER1 MB
26 dysgerminoma of ovary 9.8 DICER1 WT1
27 muscle cancer 9.8 MB WT1
28 sex cord-gonadal stromal tumor 9.8 DICER1 WT1
29 rhabdoid cancer 9.8 BCOR MB WT1
30 ewing sarcoma 9.7
31 small cell sarcoma 9.7
32 differentiating neuroblastoma 9.7
33 spindle cell sarcoma 9.7
34 myeloid sarcoma 9.7
35 dicer1-related disorders 9.7
36 dicer1-related pleuropulmonary blastoma cancer predisposition syndrome 9.7
37 embryonal sarcoma 9.7
38 horseshoe kidney 9.7
39 rhabdomyosarcoma 2 9.7 MB WT1
40 renal cell carcinoma, nonpapillary 9.6
41 islet cell tumor 9.6
42 primary amebic meningoencephalitis 9.6
43 angiomyolipoma 9.6
44 sarcomatoid renal cell carcinoma 9.6
45 hypoglycemia 9.6

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



Diseases related to Kidney Sarcoma

Symptoms & Phenotypes for Kidney Sarcoma

GenomeRNAi Phenotypes related to Kidney Sarcoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-116 9.32 BCOR
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-119 9.32 DICER1
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-145 9.32 BCOR DICER1 WT1
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-148 9.32 BCOR
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-15 9.32 DICER1
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-204 9.32 BCOR
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-40 9.32 DICER1
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-95 9.32 BCOR

MGI Mouse Phenotypes related to Kidney Sarcoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.8 B4GAT1 BCOR DICER1 MB WT1 YWHAE
2 cardiovascular system MP:0005385 9.77 BCOR DICER1 MB WT1 YWHAE
3 embryo MP:0005380 9.56 BCOR DICER1 MB WT1
4 hematopoietic system MP:0005397 9.55 B4GAT1 BCOR DICER1 MB WT1
5 mortality/aging MP:0010768 9.43 B4GAT1 BCOR DICER1 MB WT1 YWHAE
6 muscle MP:0005369 9.02 B4GAT1 DICER1 MB WT1 YWHAE

Drugs & Therapeutics for Kidney Sarcoma

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

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

Interventional clinical trials:

(show all 26)
# 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 Pencil Beam Scanning in Patients With Renal Tumors Recruiting NCT03810651 Early Phase 1
23 International PPB Registry for PPB, DICER1 and Associated Conditions Recruiting NCT03382158
24 Study of Kidney Tumors in Younger Patients Recruiting NCT00898365
25 Studying Biomarkers in Samples From Younger Patients With Kidney Cancer Withdrawn NCT01642095
26 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 Sarcoma

Genetic Tests for Kidney Sarcoma

Genetic tests related to Kidney Sarcoma:

# Genetic test Affiliating Genes
1 Renal Sarcoma 29

Anatomical Context for Kidney Sarcoma

MalaCards organs/tissues related to Kidney Sarcoma:

41
Kidney, T Cells, Lung, Ovary, Myeloid

Publications for Kidney Sarcoma

Articles related to Kidney Sarcoma:

(show all 40)
# Title Authors Year
1
Primary Low-Grade Fibromyxoid Sarcoma of Kidney-an Extremely Rare Entity. ( 29973762 )
2018
2
Delayed Metastasis of Clear Cell Sarcoma of Kidney to Bladder After 7 Disease-Free Years. ( 29509095 )
2018
3
Ewing's sarcoma of kidney in a 60-year-old patient with local recurrence: A rare occurrence. ( 30488867 )
2018
4
FNAC aided diagnosis of clear cell sarcoma of kidney: Report of two cases in infants. ( 28421721 )
2017
5
Clear cell sarcoma of kidney involving a horseshoe kidney and harboring EGFR internal tandem duplication. ( 28440018 )
2017
6
Concurrent occurrence of neuroblastoma and clear cell sarcoma of kidney in an infant: Is it a random event? ( 28195107 )
2017
7
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
8
Dual tumor - Clear cell sarcoma of kidney with differentiating neuroblastoma: A rare case with review of literature. ( 28195106 )
2017
9
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
10
Mutually exclusive BCOR internal tandem duplications and YWHAE-NUTM2 fusions in clear cell sarcoma of kidney: not the full story. ( 27000436 )
2016
11
Evolution of Renal Cysts to Anaplastic Sarcoma of Kidney in a Child With DICER1 Syndrome. ( 26928971 )
2016
12
Synovial sarcoma of kidney in a child: A rare presentation. ( 27046979 )
2016
13
Primary synovial sarcoma of kidney: a rare differential diagnosis of renomegaly. ( 25610686 )
2014
14
Clear cell sarcoma of kidney in a neonate. ( 26023506 )
2014
15
Clear cell sarcoma of kidney: morphoproteomic analysis reveals genomic correlates and therapeutic options. ( 22256788 )
2013
16
Outcomes of patients with revised stage I clear cell sarcoma of kidney treated in National Wilms Tumor Studies 1-5. ( 22658515 )
2013
17
Characterization of the chromosomal translocation t(10;17)(q22;p13) in clear cell sarcoma of kidney. ( 22294382 )
2012
18
Primary Ewing sarcoma of kidney in an elderly. ( 22797102 )
2012
19
Clear cell sarcoma of kidney: A rare entity. ( 22557783 )
2011
20
Nonleukemic granulocytic sarcoma of kidney with mixed phenotype blasts: a diagnostic dilemma. ( 21934235 )
2011
21
Primary synovial sarcoma of kidney-a report of 2 cases and review of literature. ( 21863065 )
2010
22
Primary synovial sarcoma of kidney: A rare tumor with an atypical presentation. ( 19672366 )
2009
23
Translocation (10;17)(q22;p13): a recurring translocation in clear cell sarcoma of kidney. ( 15474157 )
2004
24
Transfer of IFNgamma-depleted CD4(+) T cells together with CD8(+) T cells leads to rejection of murine kidney sarcoma in mice. ( 10925361 )
2000
25
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
26
Veno-occlusive disease-like hepatotoxicity in two children receiving chemotherapy for Wilms' tumor and clear cell sarcoma of kidney. ( 9509511 )
1998
27
Cystic embryonal sarcoma of kidney: a case report. ( 9635536 )
1998
28
Clear cell sarcoma of kidney with tumor extension into the right atrium. ( 9788798 )
1998
29
Clear cell sarcoma of kidney in an adult. ( 8149160 )
1993
30
Clear cell sarcoma of kidney. Two cases in adults. ( 8453549 )
1993
31
Clear cell sarcoma of kidney: a clinicopathological study of eight cases from Malaysia. ( 1328018 )
1992
32
Clear cell sarcoma of kidney: report of the first Malaysian case. ( 1374551 )
1992
33
Nondifferentiated-type small cell sarcoma of kidney in a young woman. ( 1871901 )
1991
34
Primitive pelvic sarcoma resembling clear cell sarcoma of kidney. ( 1755102 )
1991
35
Clear cell sarcoma of kidney (CCSK)--a clinicopathological study of 4 cases. ( 2591997 )
1989
36
Case 7. Clear cell sarcoma of kidney. ( 2748484 )
1989
37
Embryonic Sarcoma of Kidney. ( 19985727 )
1927
38
SPONTANEOUS HAEMATOMA IN SARCOMA OF KIDNEY. ( 17864796 )
1923
39
VI. Sarcoma of Kidney in an Infant; Recovery after Nephrectomy. ( 17860750 )
1899
40
Sarcoma of Kidney and Lungs. ( 20748227 )
1876

Variations for Kidney Sarcoma

Expression for Kidney Sarcoma

Search GEO for disease gene expression data for Kidney Sarcoma.

Pathways for Kidney Sarcoma

Pathways related to Kidney Sarcoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.78 WT1 YWHAE
2 10.15 BCOR YWHAE

GO Terms for Kidney Sarcoma

Cellular components related to Kidney Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 8.92 B4GAT1 DICER1 MB YWHAE

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription by RNA polymerase II GO:0000122 9.13 BCOR DICER1 WT1
2 heart development GO:0007507 8.8 BCOR MB WT1

Molecular functions related to Kidney Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 histone deacetylase binding GO:0042826 8.62 BCOR YWHAE

Sources for Kidney 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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