MCID: RTR010
MIFTS: 39

Retroperitoneal Sarcoma

Categories: Cancer diseases

Aliases & Classifications for Retroperitoneal Sarcoma

MalaCards integrated aliases for Retroperitoneal Sarcoma:

Name: Retroperitoneal Sarcoma 12 14 69

Classifications:



External Ids:

Disease Ontology 12 DOID:12341
NCIt 46 C4832
SNOMED-CT 64 307219002
UMLS 69 C0585129

Summaries for Retroperitoneal Sarcoma

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

Related Diseases for Retroperitoneal Sarcoma

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



Diseases related to Retroperitoneal Sarcoma

Symptoms & Phenotypes for Retroperitoneal Sarcoma

MGI Mouse Phenotypes related to Retroperitoneal Sarcoma:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.8 BCOR CASZ1 CREBBP MDM2 SMARCB1
2 cellular MP:0005384 9.77 BCOR CASZ1 CREBBP MDM2 SMARCB1
3 growth/size/body region MP:0005378 9.73 CREBBP MDM2 PLIN1 SMARCB1 BCOR CASZ1
4 embryo MP:0005380 9.62 BCOR CREBBP MDM2 SMARCB1
5 immune system MP:0005387 9.55 BCOR CASZ1 CREBBP MDM2 SMARCB1
6 liver/biliary system MP:0005370 9.35 CASZ1 CREBBP MDM2 PLIN1 SMARCB1
7 muscle MP:0005369 9.02 CASZ1 CREBBP MDM2 PLIN1 SMARCB1

Drugs & Therapeutics for Retroperitoneal Sarcoma

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

(show all 14)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 1, 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 46835353 6436030 5284616
6
Doxil Approved June 1999 Phase 1, Phase 2 31703
7 Anti-Bacterial Agents Phase 1, Phase 2
8 Antibiotics, Antitubercular Phase 1, Phase 2
9 Topoisomerase Inhibitors Phase 1, Phase 2
10 Alkylating Agents Phase 2
11 Antifungal Agents Phase 2
12 Anti-Infective Agents Phase 2
13 Immunosuppressive Agents Phase 2
14
Cobalt 7440-48-4 104729

Interventional clinical trials:

(show all 14)

# 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 Radiation Therapy Plus Doxorubicin in Treating Patients With Resectable Primary or Recurrent Retroperitoneal Soft Tissue Sarcoma Completed NCT00004123 Phase 1 Doxorubicin hydrochloride (DOX)
11 Proton Beam Radiation Therapy in Treating Patients With Retroperitoneal Sarcoma Active, not recruiting NCT01034566 Phase 1
12 Real-Time Contrast-Enhanced Ultrasonography and Shear Wave Elastography in Predicting Treatment Response in Patients With Soft Tissue Sarcoma Recruiting NCT02384473 Early Phase 1 Definity Suspension for Injection
13 Preoperative Imaging in Retroperitoneal Sarcoma Active, not recruiting NCT01902667
14 Preoperative Proton Radiotherapy for Retroperitoneal Sarcoma Terminated NCT00901836

Search NIH Clinical Center for Retroperitoneal Sarcoma

Genetic Tests for Retroperitoneal Sarcoma

Anatomical Context for Retroperitoneal Sarcoma

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

18
The Retroperitoneal Space

MalaCards organs/tissues related to Retroperitoneal Sarcoma:

38
Kidney, Liver, Bone

Publications for Retroperitoneal Sarcoma

Articles related to Retroperitoneal Sarcoma:

(show top 50) (show all 196)
# Title Authors Year
1
Management of metastatic retroperitoneal sarcoma: a consensus approach from the Transatlantic Retroperitoneal Sarcoma Working Group (TARPSWG). ( 29432564 )
2018
2
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
3
Surgical morbidity in retroperitoneal sarcoma resection. ( 29314041 )
2018
4
Intraoperative Electron Radiation Therapy inA Retroperitoneal Sarcoma. ( 29353660 )
2018
5
Predictors of surgical quality for retroperitoneal sarcoma: Volume matters. ( 28608360 )
2017
6
<sup>The role and outcomes of palliative surgery for retroperitoneal sarcoma</sup> . ( 29284068 )
2017
7
Effect of intraoperative radiotherapy in the treatment of retroperitoneal sarcoma. ( 28074298 )
2017
8
Effect of nephrectomy for retroperitoneal sarcoma on post-operative renal function. ( 29044533 )
2017
9
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
10
Major vascular resections in retroperitoneal sarcoma. ( 29194630 )
2017
11
Importance of preoperative diagnosis for management of patients with suspected retroperitoneal sarcoma. ( 28768365 )
2017
12
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
13
Needle tract seeding following core biopsies in retroperitoneal sarcoma. ( 28754227 )
2017
14
The modified frailty index to predict morbidity and mortality for retroperitoneal sarcoma resections. ( 28587892 )
2017
15
Clinical benefit and residual kidney function of en bloc nephrectomy for perirenal retroperitoneal sarcoma. ( 29044883 )
2017
16
Systemic therapy in retroperitoneal sarcoma management. ( 29194633 )
2017
17
Radiation therapy in retroperitoneal sarcoma management. ( 29165813 )
2017
18
Treatment of retroperitoneal sarcoma: current standards and new developments. ( 28509807 )
2017
19
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
20
Long-Term Quality of Life of Retroperitoneal Sarcoma Patients Treated with Pre-Operative Radiotherapy and Surgery. ( 29234568 )
2017
21
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
22
Retroperitoneal Sarcoma: Setting the Stage for Treatment Strategies Tailored to Histologic Subtype and Other Patient and Tumor Factors. ( 28871972 )
2017
23
Incidence and Adverse Prognostic Implications of Histopathologic Organ Invasion in Primary Retroperitoneal Sarcoma. ( 28147251 )
2017
24
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
25
Post-relapse outcomes after primary extended resection of retroperitoneal sarcoma: A report from the Trans-Atlantic RPS Working Group. ( 28152173 )
2017
26
Historical perspectives and future directions in the surgical management of retroperitoneal sarcoma. ( 29127700 )
2017
27
Postoperative Morbidity After Radical Resection of Primary Retroperitoneal Sarcoma: A Report From the Transatlantic RPS Working Group. ( 28394870 )
2017
28
The importance of surgical margins in retroperitoneal sarcoma. ( 26707028 )
2016
29
Retroperitoneal Sarcoma: Fact, Opinion, and Controversy. ( 27591493 )
2016
30
Spilled gallstones mimicking a retroperitoneal sarcoma following laparoscopic cholecystectomy. ( 27158213 )
2016
31
CT-guided (125)I seed implantation for inoperable retroperitoneal sarcoma: A technique for delivery of local tumor brachytherapy. ( 28101168 )
2016
32
Clinical outcomes of tissue expanders on adjuvant radiotherapy of resected retroperitoneal sarcoma. ( 27428199 )
2016
33
Variability in Patterns of Recurrence After Resection of Primary Retroperitoneal Sarcoma (RPS): A Report on 1007 Patients From the Multi-institutional Collaborative RPS Working Group. ( 26727100 )
2016
34
Guidelines for the Treatment of Recurrent Retroperitoneal Sarcoma: Are we Trying to Fit a Square Peg into a Round Hole? ( 27334225 )
2016
35
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
36
Management of Recurrent Retroperitoneal Sarcoma (RPS) in the Adult: A Consensus Approach from the Trans-Atlantic RPS Working Group. ( 27480354 )
2016
37
Practical Issues for Retroperitoneal Sarcoma. ( 27556665 )
2016
38
External validation of a multi-institutional retroperitoneal sarcoma nomogram. ( 26916507 )
2016
39
Is long-term survival possible after margin-positive resection of retroperitoneal sarcoma (RPS)? ( 27060344 )
2016
40
Predictors of improved survival for patients with retroperitoneal sarcoma. ( 27495850 )
2016
41
Defining the role of radiotherapy for retroperitoneal sarcoma. ( 27210905 )
2016
42
Giant Retroperitoneal Sarcoma. ( 27613734 )
2016
43
Clinical outcomes of tissue expanders on adjuvant radiotherapy of resected retroperitoneal sarcoma. ( 27755478 )
2016
44
Results of Resection for Recurrent or Residual Retroperitoneal Sarcoma After Failed Primary Treatment. ( 27554502 )
2016
45
Surgical outcome and patterns of recurrence for retroperitoneal sarcoma at a single centre. ( 26876538 )
2016
46
Hepatitis B Reactivation After Ifosfamide Therapy for Retroperitoneal Sarcoma. ( 27246626 )
2016
47
Neoadjuvant radiotherapy for retroperitoneal sarcoma: A systematic review. ( 26990903 )
2016
48
The Trans-Atlantic Retroperitoneal Sarcoma Working Group (TARPSWG): "Red wine or white"? ( 27613555 )
2016
49
Retroperitoneal Sarcoma (RPS) High Risk Gross Tumor Volume Boost (HR GTV Boost) Contour Delineation Agreement Among NRG Sarcoma Radiation and Surgical Oncologists. ( 26018727 )
2015
50
Analysis of perioperative radiation therapy in the surgical treatment of primary and recurrent retroperitoneal sarcoma. ( 26238282 )
2015

Variations for Retroperitoneal Sarcoma

Expression for Retroperitoneal Sarcoma

Search GEO for disease gene expression data for Retroperitoneal Sarcoma.

Pathways for Retroperitoneal Sarcoma

GO Terms for Retroperitoneal Sarcoma

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

# Name GO ID Score Top Affiliating Genes
1 protein complex assembly GO:0006461 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

Sources for Retroperitoneal Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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