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MCID: RTR010
MIFTS: 38

Retroperitoneal Sarcoma

Categories: Cancer diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Retroperitoneal Sarcoma

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MalaCards integrated aliases for Retroperitoneal Sarcoma:

Name: Retroperitoneal Sarcoma 12 15 73

Classifications:

MalaCards categories:
Global: Cancer diseases
See all MalaCards categories (disease lists)


External Ids:

Disease Ontology 12 DOID:12341
NCIt 50 C4832
SNOMED-CT 68 307219002
UMLS 73 C0585129
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Summaries for Retroperitoneal Sarcoma

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Disease Ontology : 12 A malignant retroperitoneal cancer and sarcoma that is located in the retroperitoneal space.

MalaCards based summary : Retroperitoneal Sarcoma is related to sarcoma and epithelial-myoepithelial carcinoma. An important gene associated with Retroperitoneal Sarcoma is CASZ1 (Castor Zinc Finger 1), and among its related pathways/superpathways are Thyroid hormone signaling pathway and Androgen receptor signaling pathway. The drugs Doxorubicin and Trabectedin have been mentioned in the context of this disorder. Affiliated tissues include the retroperitoneal space, kidney and liver, and related phenotypes are cardiovascular system and cellular

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Related Diseases for Retroperitoneal Sarcoma

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

(show all 31)
# Related Disease Score Top Affiliating Genes
1 sarcoma 10.8
2 epithelial-myoepithelial carcinoma 10.0 PLIN1 SMARCB1
3 cerebellar liponeurocytoma 10.0 PLIN1 SMARCB1
4 angiomyolipoma 9.8
5 spindle cell liposarcoma 9.7 MDM2 SMARCB1
6 renal cell carcinoma, nonpapillary 9.6
7 alveolar soft part sarcoma 9.6
8 hepatitis 9.6
9 hepatitis b 9.6
10 microphthalmia 9.6
11 varicocele 9.6
12 leiomyoma 9.6
13 cervicitis 9.6
14 adenocarcinoma 9.6
15 liposarcoma 9.6
16 cervical adenocarcinoma 9.6
17 kidney angiomyolipoma 9.6
18 hypoglycemia 9.6
19 malakoplakia 9.6
20 soft tissue sarcoma 9.6
21 ovarian fibrothecoma 9.6
22 dedifferentiated liposarcoma 9.6
23 retroperitoneum carcinoma 9.6 CASZ1 CREBBP SMARCB1
24 uterine sarcoma 9.6 MDM2 SMARCB1
25 conventional central osteosarcoma 9.5 BCOR MDM2
26 connective tissue cancer 9.5 MDM2 SMARCB1
27 bone osteosarcoma 9.5 BCOR MDM2
28 nervous system cancer 9.4 MDM2 SMARCB1
29 malignant peripheral nerve sheath tumor 9.3 MDM2 SMARCB1
30 small cell sarcoma 9.0 BCOR MDM2 SMARCB1
31 trehalase deficiency 8.7 BCOR CREBBP SMARCB1

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



Diseases related to Retroperitoneal Sarcoma
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Symptoms & Phenotypes for Retroperitoneal Sarcoma

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MGI Mouse Phenotypes related to Retroperitoneal Sarcoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.83 CASZ1 CREBBP MDM2 BCOR SMARCB1
2 cellular MP:0005384 9.8 SMARCB1 CASZ1 CREBBP MDM2 BCOR
3 growth/size/body region MP:0005378 9.8 CREBBP MDM2 BCOR PLIN1 SMARCB1 CASZ1
4 embryo MP:0005380 9.67 SMARCB1 CREBBP MDM2 BCOR
5 immune system MP:0005387 9.65 CASZ1 CREBBP MDM2 BCOR SMARCB1
6 integument MP:0010771 9.46 CASZ1 CREBBP MDM2 PLIN1
7 liver/biliary system MP:0005370 9.35 CREBBP MDM2 PLIN1 SMARCB1 CASZ1
8 muscle MP:0005369 9.02 CREBBP MDM2 PLIN1 SMARCB1 CASZ1
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Drugs & Therapeutics for Retroperitoneal Sarcoma

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

(show all 15)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 1, Phase 2,Phase 2 23214-92-8 31703
2
Trabectedin Approved, Investigational Phase 2 114899-77-3 108150
3
Everolimus Approved Phase 2 159351-69-6 6442177
4
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
5
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
6
Doxil Approved June 1999 Phase 1, Phase 2,Phase 2 31703
7 Anti-Bacterial Agents Phase 1, Phase 2,Phase 2
8 Antibiotics, Antitubercular Phase 1, Phase 2,Phase 2
9 Topoisomerase Inhibitors Phase 1, Phase 2,Phase 2
10 Alkylating Agents Phase 2
11 Antineoplastic Agents, Alkylating Phase 2
12 Antifungal Agents Phase 2
13 Anti-Infective Agents Phase 2
14 Immunosuppressive Agents Phase 2
15
Cobalt Not Applicable 7440-48-4 104729

Interventional clinical trials:

(show all 15)
# Name Status NCT ID Phase Drugs
1 A Prospective Randomized Trial of Pre-Operative IMRT+Surgery Versus Surgery Alone For Primary Retroperitoneal Sarcoma Completed NCT00131898 Phase 3
2 Surgery With or Without Radiation Therapy in Treating Patients With Primary Soft Tissue Sarcoma of the Retroperitoneum or Pelvis Completed NCT00091351 Phase 3
3 Surgery With or Without Radiation Therapy in Untreated Nonmetastatic Retroperitoneal Sarcoma Recruiting NCT01344018 Phase 3
4 Trial of Nilotinib and Adriamycin as Treatment in Liposarcomas and Leiomyosarcomas of Retroperitoneum Unknown status NCT02587169 Phase 1, Phase 2 Nilotinib-adriamycin
5 Proton or Photon RT for Retroperitoneal Sarcomas Recruiting NCT01659203 Phase 1, Phase 2
6 Efficacy Study on Trabectedin in Retroperitoneal Leiomyosarcoma and Well Differentiated/Dedifferentiated Liposarcoma Recruiting NCT02247544 Phase 2 Trabectedin
7 High Precision RT For Soft-Tissue Sarcoma Active, not recruiting NCT01389050 Phase 2
8 Study of RAD001 in Soft Tissue Extremity and/or Retroperitoneal Sarcomas Terminated NCT01048723 Phase 2 RAD001
9 Combination Chemotherapy, Radiation Therapy, and Surgery in Treating Patients With Primary or Recurrent Sarcoma Withdrawn NCT00017160 Phase 2 doxorubicin;filgrastim;ifosfamide
10 Proton Beam Radiation Therapy in Treating Patients With Retroperitoneal Sarcoma Unknown status NCT01034566 Phase 1
11 Radiation Therapy Plus Doxorubicin in Treating Patients With Resectable Primary or Recurrent Retroperitoneal Soft Tissue Sarcoma Completed NCT00004123 Phase 1 Doxorubicin hydrochloride (DOX)
12 Preoperative Imaging in Retroperitoneal Sarcoma Active, not recruiting NCT01902667
13 Long Term Morbidity and Quality of Life in Retroperitoneal Sarcomas Active, not recruiting NCT03480399
14 Real-Time Contrast-Enhanced Ultrasonography and Shear Wave Elastography in Predicting Treatment Response in Patients With Soft Tissue Sarcoma Active, not recruiting NCT02384473 Early Phase 1 Definity Suspension for Injection
15 Preoperative Proton Radiotherapy for Retroperitoneal Sarcoma Terminated NCT00901836 Not Applicable

Search NIH Clinical Center for Retroperitoneal Sarcoma

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Genetic Tests for Retroperitoneal Sarcoma

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Anatomical Context for Retroperitoneal Sarcoma

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The Foundational Model of Anatomy Ontology organs/tissues related to Retroperitoneal Sarcoma:

19
The Retroperitoneal Space

MalaCards organs/tissues related to Retroperitoneal Sarcoma:

41
Kidney, Liver, Bone
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Publications for Retroperitoneal Sarcoma

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Articles related to Retroperitoneal Sarcoma:

(show top 50) (show all 204)
# Title Authors Year
1
Neoadjuvant and adjuvant strategies in retroperitoneal sarcoma. ( 29472043 )
2018
2
Intraoperative Electron Radiation Therapy inA Retroperitoneal Sarcoma. ( 29353660 )
2018
3
Surgical morbidity in retroperitoneal sarcoma resection. ( 29314041 )
2018
4
Anterior Approach to En Bloc Resection in Left-Sided Retroperitoneal Sarcoma with Adjacent Organ Involvement: A Study of 25 Patients in a Single Center. ( 29449525 )
2018
5
Effectiveness and Safety of Tissue Expander for Adjuvant Helical Tomotherapy in Curatively Resected Retroperitoneal Sarcoma. ( 29599376 )
2018
6
Unsupervised versus Supervised Identification of Prognostic Factors in Patients with Localized Retroperitoneal Sarcoma: A Data Clustering and Mahalanobis Distance Approach. ( 29850497 )
2018
7
Strategic Delay: Histology- and Biology-Driven Decision-Making in Recurrent Retroperitoneal Sarcoma. ( 29868975 )
2018
8
A contemporary analysis of radiotherapy effect in surgically treated retroperitoneal sarcoma. ( 29631934 )
2018
9
Pancreaticoduodenectomy in the surgical management of primary retroperitoneal sarcoma. ( 29452860 )
2018
10
Management of metastatic retroperitoneal sarcoma: a consensus approach from the Transatlantic Retroperitoneal Sarcoma Working Group (TARPSWG). ( 29432564 )
2018
11
Resection of retroperitoneal sarcoma en-bloc with inferior vena cava: 20 year outcomes of a single institution. ( 29878363 )
2018
12
Minimally Invasive Surgery for Retroperitoneal Sarcoma: Just Because We Can Does Not Mean We Should. ( 29948424 )
2018
13
Effect of nephrectomy for retroperitoneal sarcoma on post-operative renal function. ( 29044533 )
2017
14
Treatment of retroperitoneal sarcoma: current standards and new developments. ( 28509807 )
2017
15
Needle tract seeding following core biopsies in retroperitoneal sarcoma. ( 28754227 )
2017
16
Systemic therapy in retroperitoneal sarcoma management. ( 29194633 )
2017
17
Clinical benefit and residual kidney function of en bloc nephrectomy for perirenal retroperitoneal sarcoma. ( 29044883 )
2017
18
Radiation therapy in retroperitoneal sarcoma management. ( 29165813 )
2017
19
The modified frailty index to predict morbidity and mortality for retroperitoneal sarcoma resections. ( 28587892 )
2017
20
Retroperitoneal Sarcoma: Setting the Stage for Treatment Strategies Tailored to Histologic Subtype and Other Patient and Tumor Factors. ( 28871972 )
2017
21
Importance of preoperative diagnosis for management of patients with suspected retroperitoneal sarcoma. ( 28768365 )
2017
22
Improved local control with an aggressive strategy of preoperative (with or without intraoperative) radiation therapy combined with radical surgical resection for retroperitoneal sarcoma. ( 28127762 )
2017
23
Overall survival after resection of retroperitoneal sarcoma at academic cancer centers versus community cancer centers: An analysis of the National Cancer Data Base. ( 28943041 )
2017
24
Contemporary reappraisal of the efficacy of adjuvant chemotherapy in resected retroperitoneal sarcoma: Evidence from a nationwide clinical oncology database and review of the literature. ( 28577717 )
2017
25
Effect of intraoperative radiotherapy in the treatment of retroperitoneal sarcoma. ( 28074298 )
2017
26
Long-Term Quality of Life of Retroperitoneal Sarcoma Patients Treated with Pre-Operative Radiotherapy and Surgery. ( 29234568 )
2017
27
Post-relapse outcomes after primary extended resection of retroperitoneal sarcoma: A report from the Trans-Atlantic RPS Working Group. ( 28152173 )
2017
28
<sup>The role and outcomes of palliative surgery for retroperitoneal sarcoma</sup> . ( 29284068 )
2017
29
Performance Analysis of the American Joint Committee on Cancer 8th Edition Staging System for Retroperitoneal Sarcoma and Development of a New Staging Algorithm for Sarcoma-Specific Survival. ( 29058143 )
2017
30
Long-term Follow-up and Post-relapse Outcome of Patients with Localized Retroperitoneal Sarcoma Treated in the Italian Sarcoma Group-Soft Tissue Sarcoma (ISG-STS) Protocol 0303. ( 29043525 )
2017
31
Historical perspectives and future directions in the surgical management of retroperitoneal sarcoma. ( 29127700 )
2017
32
Postoperative Morbidity After Radical Resection of Primary Retroperitoneal Sarcoma: A Report From the Transatlantic RPS Working Group. ( 28394870 )
2017
33
Major vascular resections in retroperitoneal sarcoma. ( 29194630 )
2017
34
Incidence and Adverse Prognostic Implications of Histopathologic Organ Invasion in Primary Retroperitoneal Sarcoma. ( 28147251 )
2017
35
Predictors of surgical quality for retroperitoneal sarcoma: Volume matters. ( 28608360 )
2017
36
CT-guided (125)I seed implantation for inoperable retroperitoneal sarcoma: A technique for delivery of local tumor brachytherapy. ( 28101168 )
2016
37
Spilled gallstones mimicking a retroperitoneal sarcoma following laparoscopic cholecystectomy. ( 27158213 )
2016
38
Giant Retroperitoneal Sarcoma. ( 27613734 )
2016
39
Guidelines for the Treatment of Recurrent Retroperitoneal Sarcoma: Are we Trying to Fit a Square Peg into a Round Hole? ( 27334225 )
2016
40
Is long-term survival possible after margin-positive resection of retroperitoneal sarcoma (RPS)? ( 27060344 )
2016
41
The importance of surgical margins in retroperitoneal sarcoma. ( 26707028 )
2016
42
Results of Resection for Recurrent or Residual Retroperitoneal Sarcoma After Failed Primary Treatment. ( 27554502 )
2016
43
Defining the role of radiotherapy for retroperitoneal sarcoma. ( 27210905 )
2016
44
Preoperative or postoperative radiotherapy versus surgery alone for retroperitoneal sarcoma: a case-control, propensity score-matched analysis of a nationwide clinical oncology database. ( 27210906 )
2016
45
Predictors of improved survival for patients with retroperitoneal sarcoma. ( 27495850 )
2016
46
The Trans-Atlantic Retroperitoneal Sarcoma Working Group (TARPSWG): &amp;quot;Red wine or white&amp;quot;? ( 27613555 )
2016
47
Neoadjuvant radiotherapy for retroperitoneal sarcoma: A systematic review. ( 26990903 )
2016
48
Surgical outcome and patterns of recurrence for retroperitoneal sarcoma at a single centre. ( 26876538 )
2016
49
External validation of a multi-institutional retroperitoneal sarcoma nomogram. ( 26916507 )
2016
50
Hepatitis B Reactivation After Ifosfamide Therapy for Retroperitoneal Sarcoma. ( 27246626 )
2016
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Variations for Retroperitoneal Sarcoma

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Expression for Retroperitoneal Sarcoma

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Search GEO for disease gene expression data for Retroperitoneal Sarcoma.
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Pathways for Retroperitoneal Sarcoma

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

# Super pathways Score Top Affiliating Genes
1
Thyroid hormone signaling pathway 37
11.54 CREBBP MDM2
2
Androgen receptor signaling pathway 1
11.42 CREBBP MDM2
3
Glucocorticoid receptor regulatory network 1
11.35 CREBBP MDM2
4
Transcription Ligand-dependent activation of the ESR1/SP pathway 22
Show member pathways
 11.25 CREBBP SMARCB1
5
Pathways Affected in Adenoid Cystic Carcinoma 1
11.19 BCOR CREBBP
6
p53 pathway 1
11.09 CREBBP MDM2
7
HIF1Alpha Pathway 64
10.99 CREBBP MDM2
8
Proteolysis Putative SUMO-1 pathway 22
Show member pathways
 10.89 CREBBP MDM2
9
Regulation of Androgen receptor activity 1
10.65 CREBBP MDM2
10
Regulation of retinoblastoma protein 1
10.55 CREBBP MDM2 SMARCB1
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GO Terms for Retroperitoneal Sarcoma

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

# Name GO ID Score Top Affiliating Genes
1 protein-containing complex assembly GO:0065003 9.16 CREBBP MDM2
2 viral process GO:0016032 9.13 CREBBP MDM2 SMARCB1
3 protein destabilization GO:0031648 8.62 CREBBP MDM2

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

# Name GO ID Score Top Affiliating Genes
1 p53 binding GO:0002039 8.8 CREBBP MDM2 SMARCB1
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Sources for Retroperitoneal Sarcoma

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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
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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