MCID: SRC002
MIFTS: 41

Sarcomatoid Renal Cell Carcinoma

Categories: Cancer diseases, Nephrological diseases

Aliases & Classifications for Sarcomatoid Renal Cell Carcinoma

MalaCards integrated aliases for Sarcomatoid Renal Cell Carcinoma:

Name: Sarcomatoid Renal Cell Carcinoma 12 15 71
Renal Cell Carcinoma, Spindle Cell 12

Classifications:



External Ids:

Disease Ontology 12 DOID:4473
MeSH 44 D002292
NCIt 50 C27893
SNOMED-CT 67 128668003
UMLS 71 C1266043

Summaries for Sarcomatoid Renal Cell Carcinoma

MalaCards based summary : Sarcomatoid Renal Cell Carcinoma, also known as renal cell carcinoma, spindle cell, is related to malignant fibrous histiocytoma and liposarcoma, and has symptoms including flank pain An important gene associated with Sarcomatoid Renal Cell Carcinoma is PAX8 (Paired Box 8), and among its related pathways/superpathways are Innate Immune System and Gastric cancer. The drugs Gemcitabine and Bevacizumab have been mentioned in the context of this disorder. Affiliated tissues include endothelial, kidney and bone marrow, and related phenotypes are cellular and digestive/alimentary

Related Diseases for Sarcomatoid Renal Cell Carcinoma

Diseases related to Sarcomatoid Renal Cell Carcinoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 312)
# Related Disease Score Top Affiliating Genes
1 malignant fibrous histiocytoma 30.4 TP53 MUC1 MDM2 KIT
2 liposarcoma 30.4 TP53 PTEN MDM2
3 spindle cell sarcoma 30.2 MUC1 MDM2 KIT
4 embryonal sarcoma 30.2 TP53 MDM2 KIT
5 cystitis 30.1 TP53 KIT CDH1
6 papillary carcinoma 30.0 S100A1 PAX8 MUC1 CDH1
7 leiomyosarcoma 30.0 TP53 S100A1 MUC1 MDM2 KIT
8 kidney cancer 30.0 TP53 PTEN IFNA2 CA9
9 clear cell renal cell carcinoma 30.0 TP53 PTEN PAX8 MUC1 CDH1 CA9
10 sarcoma, synovial 29.9 MUC1 MDM2 KIT CDH1
11 renal cell carcinoma, nonpapillary 29.8 TP53 S100A1 PTEN PAX8 MUC1 KIT
12 ewing sarcoma 29.8 TP53 MDM2 KIT CDH1
13 rhabdomyosarcoma 29.7 TP53 S100A1 PTEN MDM2 KIT
14 renal cell carcinoma, papillary, 1 29.6 TP53 PTEN PAX8 KIT CA9
15 dedifferentiated liposarcoma 29.6 TP53 S100A1 PTEN MUC1 MDM2 KIT
16 gastrointestinal stromal tumor 29.5 TP53 S100A1 PTEN KIT CDH1
17 adenocarcinoma 29.2 TP53 PTEN MUC1 MDM2 KIT CDH1
18 carcinosarcoma 29.1 TP53 S100A1 PTEN PAX8 MUC1 KIT
19 bladder cancer 28.1 TP53 PTEN MUC1 MDM2 IFNA2 CGB3
20 sarcomatoid carcinoma 10.4
21 papillary tumor of the pineal region 10.4 S100A1 MUC1
22 clear cell adenoma 10.4 S100A1 PAX8
23 parachordoma 10.4 S100A1 MUC1
24 reticular perineurioma 10.3 MUC1 KIT
25 intraneural perineurioma 10.3 S100A1 MUC1
26 axillary lipoma 10.3 MUC1 KIT
27 chondroid chordoma 10.3 S100A1 MUC1
28 fibrous histiocytoma 10.3
29 adenofibroma 10.3 TP53 MUC1
30 pericardial mesothelioma 10.3 S100A1 MUC1
31 reticulum cell sarcoma 10.3 S100A1 MUC1
32 chordoid meningioma 10.3 S100A1 MUC1
33 colon leiomyosarcoma 10.3 PAX8 KIT
34 ovarian mixed germ cell neoplasm 10.3 PAX8 KIT
35 ossifying fibromyxoid tumor 10.3 S100A1 MUC1
36 pancreatic foamy gland adenocarcinoma 10.3 TP53 MUC1
37 endosalpingiosis 10.3 PAX8 MUC1
38 bile duct mucinous adenocarcinoma 10.3 TP53 MUC1
39 ovarian seromucinous carcinoma 10.3 TP53 PAX8
40 sclerosing liposarcoma 10.3 PAX8 MDM2
41 glandular cystitis 10.3 TP53 MUC1
42 mammary analogue secretory carcinoma 10.3 TP53 MUC1
43 hydronephrosis 10.3
44 biliary tract benign neoplasm 10.3 TP53 MUC1
45 gallbladder signet ring cell adenocarcinoma 10.3 MUC1 CDH1
46 malignant exocrine pancreas neoplasm 10.3 TP53 MUC1
47 intratubular embryonal carcinoma 10.3 TP53 KIT
48 ovarian serous cystadenofibroma 10.3 TP53 MUC1
49 gastric liposarcoma 10.3 MDM2 KIT
50 spindle cell rhabdomyosarcoma 10.3 S100A1 MUC1

Graphical network of the top 20 diseases related to Sarcomatoid Renal Cell Carcinoma:



Diseases related to Sarcomatoid Renal Cell Carcinoma

Symptoms & Phenotypes for Sarcomatoid Renal Cell Carcinoma

UMLS symptoms related to Sarcomatoid Renal Cell Carcinoma:


flank pain

MGI Mouse Phenotypes related to Sarcomatoid Renal Cell Carcinoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.13 CDH1 GATA3 KIT MDM2 PAX8 PTEN
2 digestive/alimentary MP:0005381 10.06 CA9 CDH1 GATA3 KIT MDM2 PTEN
3 endocrine/exocrine gland MP:0005379 10.01 CA9 CDH1 GATA3 KIT MDM2 PAX8
4 embryo MP:0005380 9.98 CDH1 GATA3 KIT MDM2 PAX8 PTEN
5 integument MP:0010771 9.91 CDH1 GATA3 KIT MDM2 PTEN RELN
6 muscle MP:0005369 9.87 GATA3 KIT MDM2 PTEN RELN S100A1
7 normal MP:0002873 9.76 CDH1 GATA3 KIT MDM2 PAX8 PTEN
8 no phenotypic analysis MP:0003012 9.73 CDH1 GATA3 KIT MDM2 PAX8 TP53
9 pigmentation MP:0001186 9.35 GATA3 KIT MDM2 PTEN TP53
10 reproductive system MP:0005389 9.23 CDH1 GATA3 KIT MDM2 PAX8 PTEN

Drugs & Therapeutics for Sarcomatoid Renal Cell Carcinoma

Drugs for Sarcomatoid Renal Cell Carcinoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 15)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Gemcitabine Approved Phase 2 95058-81-4 60750
2
Bevacizumab Approved, Investigational Phase 2 216974-75-3
3
Capecitabine Approved, Investigational Phase 2 154361-50-9 60953
4 Angiogenesis Inhibitors Phase 2
5 Antineoplastic Agents, Immunological Phase 2
6 Immunosuppressive Agents Phase 2
7 Immunologic Factors Phase 2
8 Immunoglobulins Phase 2
9 Antimetabolites Phase 2
10 Anti-Infective Agents Phase 2
11 Mitogens Phase 2
12 Antiviral Agents Phase 2
13 Antibodies Phase 2
14 Antibodies, Monoclonal Phase 2
15 Endothelial Growth Factors Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase II Safety and Efficacy Study of Capecitabine, Gemcitabine, and Bevacizumab in Combination for Patients With Metastatic or Unresectable Sarcomatoid Renal Cell Carcinoma Completed NCT00496587 Phase 2 Capecitabine;Gemcitabine;Bevacizumab

Search NIH Clinical Center for Sarcomatoid Renal Cell Carcinoma

Genetic Tests for Sarcomatoid Renal Cell Carcinoma

Anatomical Context for Sarcomatoid Renal Cell Carcinoma

MalaCards organs/tissues related to Sarcomatoid Renal Cell Carcinoma:

40
Endothelial, Kidney, Bone Marrow, Small Intestine, Colon, Neutrophil

Publications for Sarcomatoid Renal Cell Carcinoma

Articles related to Sarcomatoid Renal Cell Carcinoma:

(show top 50) (show all 240)
# Title Authors PMID Year
1
Sarcomatoid Renal Cell Carcinoma: An Immune Checkpoint-responsive Subset? 61
33593655 2021
2
Checking the Hippo in Sarcomatoid Renal Cell Carcinoma with Immunotherapy. 61
33106290 2021
3
Efficacy and Safety of Nivolumab Plus Ipilimumab versus Sunitinib in First-line Treatment of Patients with Advanced Sarcomatoid Renal Cell Carcinoma. 61
32873572 2021
4
The Great Imposter: A Confusing Case of a Rare Renal Cell Carcinoma. 61
33261232 2020
5
Sarcomatoid renal cell carcinoma: biology, natural history and management. 61
33051619 2020
6
Re: Nizar M. Tannir, Sabina Signoretti, Toni K. Choueiri, et al. Efficacy and Safety of Nivolumab Plus Ipilimumab versus Sunitinib in First-line Treatment of Patients with Advanced Sarcomatoid Renal Cell Carcinoma. Clin Cancer Res. In press. https://doi.org/10.1158/1078-0432.ccr-20-2063. 61
33121939 2020
7
Atrial Metastasis From Sarcomatoid Renal Cell Carcinoma: Integration Between 18F-FDG PET/CT and Cardiac 3-Dimensional Volume Rendering. 61
32956126 2020
8
Dermoscopy of scalp cutaneous metastasis of sarcomatoid renal cell carcinoma. 61
32794299 2020
9
Evolving Paradigms in the Management and Outcomes of Sarcomatoid Renal Cell Carcinoma in the Era of Immune Checkpoint Inhibitors. 61
33117461 2020
10
Sarcomatoid renal cell carcinoma with autosomal dominant polycystic kidney disease: a case report and literature review. 61
33064294 2020
11
Patients with sarcomatoid renal cell carcinoma - re-defining the first-line of treatment: A meta-analysis of randomised clinical trials with immune checkpoint inhibitors. 61
32712550 2020
12
[A Case of Progressive Sarcomatoid Renal Cell Carcinoma Treated with Second-Line Nivolumab after Surgery]. 61
32882123 2020
13
An unusual case of sarcomatoid renal cell carcinoma presenting in the skin by direct extension at a laparoscopic port site. 61
31998984 2020
14
Construction and validation of a novel prognostic nomogram for patients with sarcomatoid renal cell carcinoma: a SEER-based study. 61
32361824 2020
15
A Population Study to Identify Candidates for Cytoreductive Nephrectomy in Patients with Metastatic Sarcomatoid Renal Cell Carcinoma from the Surveillance, Epidemiology, and End Results (SEER) Database. 61
32516796 2020
16
MDM2-positive papillary sarcomatoid renal cell carcinoma: a potential diagnostic pitfall. 61
31732813 2020
17
Propensity-score matched comparison of partial versus radical nephrectomy for T1N0M0 sarcomatoid renal cell carcinoma. 61
32420130 2020
18
Small Renal Mass with Sarcomatoid Features. 61
32398998 2020
19
Salvage ipilimumab associated with a significant response in sarcomatoid renal cell carcinoma. 61
32114501 2020
20
Outcomes Associated with First-Line anti-PD-1/ PD-L1 agents vs. Sunitinib in Patients with Sarcomatoid Renal Cell Carcinoma: A Systematic Review and Meta-Analysis. 61
32050629 2020
21
The Role of Cytoreductive Nephrectomy for Sarcomatoid Renal Cell Carcinoma: A 29-Year Institutional Experience. 61
31726184 2020
22
Immunotherapy in sarcomatoid renal cell carcinoma: A case for optimism. 61
33307510 2020
23
Immunotherapy in metastatic sarcomatoid renal cell carcinoma: A single institution experience. 61
33310369 2020
24
A CT-Based Radiomics Approach for the Differential Diagnosis of Sarcomatoid and Clear Cell Renal Cell Carcinoma. 61
32775436 2020
25
Pemphigus in Sarcomatoid Renal Cell Carcinoma: A Rare Paraneoplastic Manifestation. 61
32165843 2020
26
The predictive role of preoperative and postoperative neutrophil-lymphocyte ratio in sarcomatoid renal cell carcinoma. 61
31590969 2019
27
A multicenter, prospective phase II trial of gemcitabine plus axitinib in patients with renal cell carcinoma with a predominant sarcomatoid component. 61
31231787 2019
28
Sarcomatoid Renal Cell Carcinoma With Unusual Metastasis to the Small Intestine Manifesting as Perforated Appendicitis. 61
31662560 2019
29
Retrospective review of experience with sarcomatoid renal cell carcinoma: Multimodality treatment remains an unmet goal. 61
31649458 2019
30
Analysis of the factors influencing the survival time of patients with sarcomatoid renal cell carcinoma. 61
31475069 2019
31
Sarcomatoid Chromophobe Renal Cell Carcinoma: A Case Report and Review of the Literature. 61
31423007 2019
32
Renal cell carcinomas with a mesenchymal stromal component: what do we know so far? 61
31266596 2019
33
Sarcomatoid Renal Cell Carcinoma: Population-Based Study of 879 Patients. 61
30799129 2019
34
Patterns of Presentation and Treatment Outcomes of Non-clear-cell Renal Cell Carcinoma and Sarcomatoid Renal Cell Carcinoma Patients in 2 Tertiary Referral Centers in Sydney, Australia. 61
30935815 2019
35
MDM2 amplification and immunohistochemical expression in sarcomatoid renal cell carcinoma. 61
30825458 2019
36
Comprehensive Genomic Profiling of Adult Renal Sarcomas Provides Insight into Disease Biology and Opportunities for Targeted Therapies. 61
31412008 2019
37
Anaplastic sarcoma of the kidney: Case report and literature review. 61
31007495 2019
38
PAX8 Expression in Solitary Fibrous Tumor: A Potential Diagnostic Pitfall. 61
28749793 2019
39
3D reconstruction of CT scans aid in preoperative planning for sarcomatoid renal cancer: A case report and mini-review. 61
30689600 2019
40
[CLINICAL CONSIDERATION OF PERCUTANEOUS RENAL BIOPSY FOR ADVANCED RENAL TUMOR]. 61
32307386 2019
41
Real-world evidence on first-line treatment for metastatic renal cell carcinoma with non-clear cell and sarcomatoid histologies: are sunitinib and pazopanib interchangeable? 61
31921344 2019
42
A Case of Sarcomatoid Renal Cell Carcinoma With Osseous Metaplasia and Papillary Renal Cell Carcinoma Metastasis. 61
31206101 2019
43
Metastatic sarcomatoid renal cell carcinoma treated with immune checkpoint inhibitors. 61
31413914 2019
44
Complete Response of a Patient With Metastatic Sarcomatoid Renal Cell Carcinoma to a Programmed Death-1 Checkpoint Inhibitor. 61
29920137 2018
45
Sarcomatoid renal cell carcinoma: Biology and treatment advances. 61
29306556 2018
46
Treatments, Outcomes, and Validity of Prognostic Scores in Patients With Sarcomatoid Renal Cell Carcinoma: A 20-Year Single-Institution Experience. 61
29395949 2018
47
Sarcomatoid renal cell carcinoma: a case report and literature review. 61
29636020 2018
48
Ki-67 index and percentage of sarcomatoid differentiation were two independent prognostic predictors in sarcomatoid renal cell carcinoma. 61
30464630 2018
49
Extensive Metastatic Sarcomatoid Renal Cell Carcinoma Evaluated by 18F-FDG PET/CT: a Case Report and Review of Literature. 61
29354391 2018
50
Rapid recurrence and radiographic progression of sarcomatoid renal cell carcinoma. 61
29034177 2018

Variations for Sarcomatoid Renal Cell Carcinoma

Expression for Sarcomatoid Renal Cell Carcinoma

Search GEO for disease gene expression data for Sarcomatoid Renal Cell Carcinoma.

Pathways for Sarcomatoid Renal Cell Carcinoma

Pathways related to Sarcomatoid Renal Cell Carcinoma according to GeneCards Suite gene sharing:

(show all 25)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.77 TP53 S100A1 PTEN MUC1 MDM2 KIT
2
Show member pathways
12.59 TP53 PTEN KIT CDH1
3
Show member pathways
12.57 TP53 RELN PTEN MDM2 KIT IFNA2
4
Show member pathways
12.53 TP53 PTEN MDM2 KIT
5
Show member pathways
12.48 TP53 PTEN MDM2 CDH1
6
Show member pathways
12.18 TP53 PTEN PAX8 MDM2 KIT CDH1
7
Show member pathways
12.12 TP53 PTEN MDM2 GATA3
8
Show member pathways
12.02 TP53 PTEN MUC1 MDM2 CDH1
9 11.99 TP53 IFNA2 GATA3
10 11.98 TP53 PTEN MDM2
11 11.89 TP53 PTEN PAX8 MDM2 KIT IFNA2
12 11.88 TP53 PTEN MDM2
13 11.81 TP53 PTEN MDM2 CDH1
14 11.8 TP53 PTEN MDM2
15 11.79 TP53 MUC1 GATA3
16 11.77 TP53 PTEN MDM2
17 11.74 TP53 PTEN MDM2
18 11.6 TP53 MDM2 GATA3
19 11.54 KIT IFNA2 CDH1
20 11.52 TP53 MDM2 CDH1
21 11.48 TP53 PTEN KIT
22 11.31 TP53 PTEN MDM2
23 11.21 TP53 PTEN MDM2
24
Show member pathways
10.95 TP53 MDM2
25 10.94 TP53 MDM2

GO Terms for Sarcomatoid Renal Cell Carcinoma

Cellular components related to Sarcomatoid Renal Cell Carcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytoplasmic side of plasma membrane GO:0009898 8.8 PTEN KIT CDH1

Biological processes related to Sarcomatoid Renal Cell Carcinoma according to GeneCards Suite gene sharing:

(show all 23)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription, DNA-templated GO:0045893 9.95 TP53 PAX8 IFNA2 GATA3 CDH1
2 apoptotic process GO:0006915 9.93 TP53 PTEN MDM2 IFNA2 CGB3
3 negative regulation of gene expression GO:0010629 9.83 TP53 MDM2 IFNA2 GATA3
4 positive regulation of gene expression GO:0010628 9.83 TP53 PTEN MDM2 KIT GATA3
5 protein deubiquitination GO:0016579 9.81 TP53 PTEN MDM2 GATA3
6 cellular response to hypoxia GO:0071456 9.76 TP53 PTEN MDM2
7 regulation of neuron apoptotic process GO:0043523 9.61 TP53 GATA3
8 positive regulation of protein export from nucleus GO:0046827 9.61 TP53 MDM2
9 embryonic hemopoiesis GO:0035162 9.6 KIT GATA3
10 positive regulation of cardiac muscle cell apoptotic process GO:0010666 9.59 TP53 PTEN
11 DNA damage response, signal transduction by p53 class mediator resulting in transcription of p21 class mediator GO:0006978 9.58 TP53 MUC1
12 DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest GO:0006977 9.58 TP53 MUC1 MDM2
13 cardiac septum morphogenesis GO:0060411 9.57 TP53 MDM2
14 mesonephros development GO:0001823 9.56 PAX8 GATA3
15 otic vesicle development GO:0071599 9.49 PAX8 GATA3
16 cellular response to UV-C GO:0071494 9.48 TP53 MDM2
17 positive regulation of transcription from RNA polymerase II promoter in response to stress GO:0036003 9.46 TP53 MUC1
18 central nervous system development GO:0007417 9.46 TP53 RELN PTEN PAX8
19 positive regulation of thyroid hormone generation GO:2000611 9.37 PAX8 GATA3
20 cytokine-mediated signaling pathway GO:0019221 9.35 TP53 MUC1 KIT IFNA2 GATA3
21 cellular response to actinomycin D GO:0072717 9.32 TP53 MDM2
22 mast cell differentiation GO:0060374 9.26 KIT GATA3
23 response to drug GO:0042493 9.1 TP53 PTEN MDM2 GATA3 CDH1 CA9

Molecular functions related to Sarcomatoid Renal Cell Carcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 DNA-binding transcription activator activity, RNA polymerase II-specific GO:0001228 9.26 TP53 PAX8 MDM2 GATA3
2 p53 binding GO:0002039 8.8 TP53 MUC1 MDM2

Sources for Sarcomatoid Renal Cell Carcinoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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