MCID: RTR010
MIFTS: 35

Retroperitoneal Sarcoma

Categories: Cancer diseases

Aliases & Classifications for Retroperitoneal Sarcoma

MalaCards integrated aliases for Retroperitoneal Sarcoma:

Name: Retroperitoneal Sarcoma 12 15 70

Classifications:



External Ids:

Disease Ontology 12 DOID:12341
NCIt 50 C4832
SNOMED-CT 67 307219002
UMLS 70 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 spindle cell sarcoma and sarcoma. An important gene associated with Retroperitoneal Sarcoma is RPRD1B (Regulation Of Nuclear Pre-MRNA Domain Containing 1B), and among its related pathways/superpathways is Mesenchymal Stem Cell Differentiation Pathways and Lineage-specific Markers. The drugs Bupivacaine and Everolimus have been mentioned in the context of this disorder. Affiliated tissues include retroperitoneal space, kidney and bone, and related phenotypes are integument and muscle

Related Diseases for Retroperitoneal Sarcoma

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

(show top 50) (show all 287)
# Related Disease Score Top Affiliating Genes
1 spindle cell sarcoma 30.5 SSX2 MYOG MDM2 KIT CD34 CALD1
2 sarcoma 30.4 SSX2 SMARCB1 MYOG MDM2 KIT CDK4
3 leiomyosarcoma 30.3 MYOG MLANA MDM2 KIT CDK4
4 malignant fibrous histiocytoma 30.2 MYOG MDM2 CDK4
5 liposarcoma 30.1 TSPAN31 MYOG MDM2 CDK4 CD34
6 embryonal rhabdomyosarcoma 29.8 MYOG MDM2 CDK4
7 neurilemmoma 29.6 SMARCB1 KIT CD34 ANO1
8 pleomorphic lipoma 29.4 TSPAN31 MDM2 CDK4 CD34
9 embryonal sarcoma 29.3 MYOG MDM2 KIT CDK4
10 retroperitoneal leiomyosarcoma 29.3 RPRD1B RPRD1A KIT CD34 CALD1 ANO1
11 ewing sarcoma 29.3 MYOG MDM2 KIT CDK4
12 rhabdomyosarcoma 28.8 SSX2 SMARCB1 MYOG MDM2 KIT CDK4
13 dedifferentiated liposarcoma 28.7 TSPAN31 SSX2 MYOG MDM2 KIT CDK4
14 sarcoma, synovial 28.4 SSX2 SMARCB1 MYOG MDM2 KIT CD34
15 well-differentiated liposarcoma 28.4 TSPAN31 SSX2 MYOG MLANA MDM2 KIT
16 lipomatosis, multiple 28.3 TSPAN31 MLANA MDM2 KIT CDK4 CD34
17 soft tissue sarcoma 10.6
18 ring chromosome 7 10.3 MDM2 CDK4
19 urethra leiomyoma 10.3 KIT CALD1
20 ring chromosome 10.3 MDM2 CDK4
21 mediastinum liposarcoma 10.3 MDM2 CDK4
22 glomangioma 10.3 CD34 CALD1
23 diffuse lipomatosis 10.3 MDM2 CDK4
24 lipoblastoma 10.3 MDM2 CDK4
25 malignant inflammatory fibrous histiocytoma 10.3 MDM2 CDK4
26 pancreas sarcoma 10.3 MDM2 CDK4
27 aortic malignant tumor 10.3 MDM2 CD34
28 adult liposarcoma 10.3 MDM2 CDK4
29 ovarian melanoma 10.3 MLANA CALD1
30 sclerosing liposarcoma 10.3 MDM2 CD34
31 intravascular angioleiomyoma 10.3 CD34 CALD1
32 juxtacortical osteosarcoma 10.3 MDM2 CDK4
33 breast myofibroblastoma 10.3 CD34 CALD1
34 pulmonary vein leiomyosarcoma 10.3 CD34 CALD1
35 spinal cord sarcoma 10.3 KIT CD34
36 glomangiomyoma 10.3 CD34 CALD1
37 gallbladder leiomyoma 10.3 KIT CD34
38 malignant anus melanoma 10.3 MLANA KIT
39 inflammatory liposarcoma 10.3 MDM2 CD34
40 peritoneal benign neoplasm 10.3 CD34 CALD1
41 neuronal intestinal dysplasia, type b 10.3 KIT CD34
42 malignant dermis tumor 10.3 MLANA KIT
43 mucocele of appendix 10.3 KIT CD34
44 malignant skin fibrous histiocytoma 10.3 MLANA KIT
45 benign fibrous mesothelioma 10.3 KIT CD34
46 retroperitoneal hemangiopericytoma 10.3 KIT CD34
47 vulvar melanoma 10.3 MLANA KIT
48 uterus perivascular epithelioid cell tumor 10.3 MLANA CALD1
49 penis sarcoma 10.3 MLANA CD34
50 testicular fibroma 10.3 CD34 CALD1

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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 integument MP:0010771 9.56 ANO1 CD34 CDK4 KIT MDM2 MLANA
2 muscle MP:0005369 9.1 ANO1 CDK4 KIT MDM2 MYOG SMARCB1

Drugs & Therapeutics for Retroperitoneal Sarcoma

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

(show all 8)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bupivacaine Approved, Investigational Phase 2 2180-92-9, 38396-39-3 2474
2
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
3 Analgesics, Non-Narcotic Phase 2
4 Anesthetics Phase 2
5 Analgesics, Opioid Phase 2
6 Anesthetics, Local Phase 2
7 Immunosuppressive Agents Phase 2
8
Cobalt Approved, Experimental 7440-48-4 104729

Interventional clinical trials:

(show all 18)
# Name Status NCT ID Phase Drugs
1 A Phase III Randomized Study of Preoperative Radiotherapy Plus Surgery Versus Surgery Alone for Patients With Retroperitoneal Sarcoma (RPS) Unknown status NCT01344018 Phase 3
2 Phase III Prospective Randomized Trial of Pre-Operative Intensity Modulated Radiation Therapy (IMRT) Plus Surgery Versus Surgery Alone For Primary Retroperitoneal Sarcoma Completed NCT00131898 Phase 3
3 A Phase III Randomized Study of Preoperative Radiation Plus Surgery Versus Surgery Alone for Patients With Retroperitoneal Sarcomas (RPS) Completed NCT00091351 Phase 3
4 A Randomized Phase III Study of Neoadjuvant Chemotherapy Followed by Surgery Versus Surgery Alone for Patients With High Risk RetroPeritoneal Sarcoma (RPS) Recruiting NCT04031677 Phase 3 Preoperative chemotherapy
5 Phase I/II Trial of Pre-Operative Image Guided Intensity Modulated Proton Radiation Therapy (IMPT) or Photon (IMRT) With Simultaneously Integrated Boost to the High Risk Margin for Retroperitoneal Sarcomas Recruiting NCT01659203 Phase 1, Phase 2
6 Randomized Controlled Trial of Repeat vs. Single Quadratus Lumborum Block to Reduce Opioid Prescriptions After Open Resection of Retroperitoneal Sarcoma ("RESQU-SARC" Trial) Recruiting NCT04189783 Phase 2 Liposomal Bupivacaine;Quadratus Lumborum Block
7 Use of High Precision Radiotherapy in the Management of Soft-Tissue Sarcomas Active, not recruiting NCT01389050 Phase 2
8 A Single-arm, Open Label Phase II Study of RAD001 in Soft Tissue Extremity and/or Retroperitoneal Sarcomas Terminated NCT01048723 Phase 2 RAD001
9 Phase II Study of Neoadjuvant Doxorubicin and Ifosfamide, Radiotherapy, and Surgical Resection in Patients With Primary or Recurrent Retroperitoneal Sarcoma Withdrawn NCT00017160 Phase 2 doxorubicin;filgrastim;ifosfamide
10 A Phase I Study of Preoperative Chemoradiation and Intraoperative Radiation Therapy in the Treatment of Retroperitoneal Sarcoma Completed NCT00004123 Phase 1 Doxorubicin hydrochloride (DOX)
11 Radical Resection and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in the Treatment of High Risk Recurrent Retroperitoneal Sarcoma Unknown status NCT03792867 Doxorubicin
12 Perioperative Nutritional and Hemodinamic Support in Retroperitoneal Sarcoma Patients. A Phase II Prospective Feasibility Study. Completed NCT03877588
13 Preoperative Imaging in Retroperitoneal Sarcoma Completed NCT01902667
14 REtroperitoneal SArcoma Registry (RESAR): Prospective Collection of Primary Retroperitoneal Sarcoma Patient's Data, Radiological and Pathological Material for the TransAtlantic Retroperitoneal Sarcoma Working Group Recruiting NCT03838718
15 Long Term Morbidity and Quality of Life After Multivisceral Resection for Primary Retroperitoneal Soft Tissue Sarcomas: a Prospective Observational Study Active, not recruiting NCT03480399
16 The PET-RPS Study: Utility of PET for Assessment of Response to Preoperative Chemotherapy in Primary Retroperitoneal Sarcoma (RPS) Not yet recruiting NCT04224948
17 A Pilot Study Investigating Preoperative Proton Radiotherapy for Retroperitoneal Sarcoma Terminated NCT00901836
18 Proton Radiotherapy for Retroperitoneal Sarcoma Withdrawn NCT01034566

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:

19
Retroperitoneal Space

MalaCards organs/tissues related to Retroperitoneal Sarcoma:

40
Kidney, Bone, Prostate, Smooth Muscle, Small Intestine, Pancreas

Publications for Retroperitoneal Sarcoma

Articles related to Retroperitoneal Sarcoma:

(show top 50) (show all 545)
# Title Authors PMID Year
1
Defining the role of neoadjuvant systemic therapy in high-risk retroperitoneal sarcoma: A multi-institutional study from the Transatlantic Australasian Retroperitoneal Sarcoma Working Group. 61
33206381 2021
2
Renal Function After Retroperitoneal Sarcoma Resection with Nephrectomy: A Matched Analysis of the United States Sarcoma Collaborative Database. 61
33146839 2021
3
Morbidity and Outcomes After Distal Pancreatectomy for Primary Retroperitoneal Sarcoma: An Analysis by the Trans-Atlantic Australasian Retroperitoneal Sarcoma Working Group. 61
33740198 2021
4
ASO Author Reflections: Kidney Function After Retroperitoneal Sarcoma Resection with Nephrectomy. 61
33146840 2021
5
[Preoperative radiotherapy plus resection versus surgery alone in patients with primary retroperitoneal sarcoma (EORTC-62092: STRASS): a multicenter randomized phase III study]. 61
33403443 2021
6
ASO Author Reflections: Every Step Counts: Improved Survival of Retroperitoneal Sarcoma Patients During the Past 15 Years. 61
33057862 2021
7
The importance of the margin of resection and external radiation in non-lipomatous retroperitoneal sarcoma. 61
33213828 2021
8
Primary mesenteric sarcomas: Collaborative experience from the Trans-Atlantic Australasian Retroperitoneal Sarcoma Working Group (TARPSWG). 61
33368277 2021
9
Splenic artery dissection: an unusual clinical presentation mimicking a retroperitoneal sarcoma. 61
32621626 2021
10
Nephrectomy for Retroperitoneal Sarcoma: Stay Calm and (Cautiously) Carry On. 61
33150550 2021
11
Has the Outcome for Patients Who Undergo Resection of Primary Retroperitoneal Sarcoma Changed Over Time? A Study of Time Trends During the Past 15 years. 61
33073340 2021
12
International Collaboration for the Treatment of Recurrent Retroperitoneal Sarcoma: What Have We Learned from Trying to Fit a Square Peg in a Round Hole? 61
33660130 2021
13
The importance of the margin of resection and radiotherapy in retroperitoneal liposarcoma. 61
33256943 2021
14
Risk Assessment of Visceral Sarcomas: A Comparative Study of 2698 Cases from the SEER Database. 61
33538930 2021
15
ASO Author Reflections: Preoperative Biopsy for Retroperitoneal Sarcoma. 61
33638039 2021
16
Distal Pancreatectomy for Primary Retroperitoneal Sarcoma-Clinical Implications and Future Directions. 61
33638045 2021
17
The impact of postoperative radiological surveillance intensity on disease free and overall survival from primary retroperitoneal, abdominal and pelvic soft-tissue sarcoma. 61
33549374 2021
18
Preoperative Biopsy in Patients with Retroperitoneal Sarcoma: Usage and Outcomes in a National Cohort. 61
33591480 2021
19
Postoperative Morbidity After Resection of Recurrent Retroperitoneal Sarcoma: A Report from the Transatlantic Australasian RPS Working Group (TARPSWG). 61
33389288 2021
20
Perioperative blood transfusion is not an independent predictor for worse outcomes in retroperitoneal sarcoma surgery. 61
33483237 2021
21
Preoperative radiotherapy for retroperitoneal sarcoma. 61
33387501 2021
22
Preoperative radiotherapy for retroperitoneal sarcoma. 61
33387499 2021
23
Preoperative radiotherapy for retroperitoneal sarcoma - Authors' reply. 61
33387505 2021
24
Preoperative radiotherapy for retroperitoneal sarcoma. 61
33387503 2021
25
Strategies for care of patients with gastrointestinal stromal tumor or soft tissue sarcoma during COVID-19 pandemic: A guide for surgical oncologists. 61
33022754 2021
26
Aggressive and frequent resection of recurrences in retroperitoneal sarcoma contributes to long-term survival. 61
33449172 2021
27
What is the role of preoperative radiation in primary retroperitoneal sarcoma? 61
33342550 2020
28
Post-Operative Superior Mesenteric Artery Syndrome Following Retroperitoneal Sarcoma Resection. 61
33599214 2020
29
Innate inflammatory markers for predicting survival in retroperitoneal sarcoma. 61
32808301 2020
30
Retroperitoneal tumors: Review of diagnosis and management. 61
32914475 2020
31
Multidisciplinary Oncovascular Surgery is Safe and Effective in the Treatment of Intra-abdominal and Retroperitoneal Sarcomas: A Retrospective Single Centre Cohort Study and a Comprehensive Literature Review. 61
32741678 2020
32
Analysis of textbook outcomes among patients undergoing resection of retroperitoneal sarcoma: A multi-institutional analysis of the US Sarcoma Collaborative. 61
32696475 2020
33
Enhanced recovery after surgery pathway in patients with soft tissue sarcoma. 61
32618371 2020
34
Patterns of recurrence and survival probability after second recurrence of retroperitoneal sarcoma: A study from TARPSWG. 61
32797703 2020
35
A Prospective Observational Study of Multivisceral Resection for Retroperitoneal Sarcoma: Clinical and Patient-Reported Outcomes 1 Year After Surgery. 61
33175262 2020
36
ASO Author Reflections: Understanding the Impact of Primary Retroperitoneal Sarcoma Resection on Patient Well-being: One Step Forward in Learning How Caring for Patients is as Important as Curing Their Cancers. 61
33249522 2020
37
Applicability of the Sarculator and MSKCC nomograms to retroperitoneal sarcoma prognostication in an Asian tertiary center. 61
31941593 2020
38
Response to Central Boost Radiation Therapy in an Unresectable Retroperitoneal Sarcoma: A Case Report. 61
33305102 2020
39
[French ccAFU guidelines - update 2020-2022: retroperitoneal sarcoma]. 61
33349428 2020
40
Postoperative Outcome of Surgery with Pancreatic Resection for Retroperitoneal Soft Tissue Sarcoma: Results of a Retrospective Bicentric Analysis on 50 Consecutive Patients. 61
33236323 2020
41
[A Case of Abdominal Fullness Caused by a Retroperitoneal Bulky Tumor Treated with Epidural Analgesia]. 61
33268739 2020
42
Integrative assessment of clinicopathological parameters and the expression of PD-L1, PD-L2 and PD-1 in tumor cells of retroperitoneal sarcoma. 61
32952659 2020
43
Evaluation of Preoperative Chemotherapy or Radiation and Overall Survival in Patients With Nonmetastatic, Resectable Retroperitoneal Sarcoma. 61
33175172 2020
44
Preoperative radiotherapy plus surgery versus surgery alone for patients with primary retroperitoneal sarcoma (EORTC-62092: STRASS): a multicentre, open-label, randomised, phase 3 trial. 61
32941794 2020
45
Improved Biopsy Accuracy in Retroperitoneal Dedifferentiated Liposarcoma. 61
32367501 2020
46
Primary localized retroperitoneal sarcomas: report from Slovenian sarcoma referral center. 61
33109203 2020
47
Clinical Impact of Tumor-Infiltrating Lymphocytes and PD-L1-Positive Cells as Prognostic and Predictive Biomarkers in Urological Malignancies and Retroperitoneal Sarcoma. 61
33121123 2020
48
Discovery of a duplicated inferior vena cava during surgical resection for retroperitoneal sarcoma. 61
32513629 2020
49
Severe Postoperative Complications are Associated with Impaired Survival in Primary but not in Recurrent Retroperitoneal Sarcoma. 61
33025356 2020
50
Virtual Biobanking for Retroperitoneal Sarcoma: A Transatlantic Australasian Retroperitoneal Sarcoma Working Group (TARPSWG) Initiative. 61
32591952 2020

Variations for Retroperitoneal Sarcoma

Expression for Retroperitoneal Sarcoma

Search GEO for disease gene expression data for Retroperitoneal Sarcoma.

Pathways for Retroperitoneal Sarcoma

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

# Super pathways Score Top Affiliating Genes
1 10.78 MYOG KIT CD34

GO Terms for Retroperitoneal Sarcoma

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

# Name GO ID Score Top Affiliating Genes
1 RNA polymerase II, holoenzyme GO:0016591 8.62 RPRD1B RPRD1A

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of muscle cell differentiation GO:0051149 9.26 MYOG MDM2
2 negative regulation of cell cycle arrest GO:0071157 9.16 MDM2 CDK4
3 positive regulation of cell proliferation GO:0008284 9.02 TSPAN31 RPRD1B MDM2 KIT CDK4
4 dephosphorylation of RNA polymerase II C-terminal domain GO:0070940 8.96 RPRD1B RPRD1A

Sources for Retroperitoneal Sarcoma

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-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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