MCID: RTR010
MIFTS: 34

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 47 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 large intestine lipoma. 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 Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include kidney, liver and bone, and related phenotypes are cardiovascular system and embryo

Related Diseases for Retroperitoneal Sarcoma

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

(show all 26)
id Related Disease Score Top Affiliating Genes
1 sarcoma 10.7
2 large intestine lipoma 9.9 PLIN1 SMARCB1
3 angiomyolipoma 9.7
4 cutaneous liposarcoma 9.7 MDM2 SMARCB1
5 ascaridiasis 9.6 MDM2 SMARCB1
6 fetishism 9.6 CASZ1 CREBBP SMARCB1
7 small cell sarcoma 9.6 MDM2 SMARCB1
8 microphthalmia 9.5
9 varicocele 9.5
10 leiomyoma 9.5
11 cervicitis 9.5
12 adenocarcinoma 9.5
13 liposarcoma 9.5
14 cervical adenocarcinoma 9.5
15 hypoglycemia 9.5
16 hemihypertrophy 9.5
17 malakoplakia 9.5
18 renal cell carcinoma 9.5
19 ovarian fibrothecoma 9.5
20 alveolar soft-part sarcoma 9.5
21 dedifferentiated liposarcoma 9.5
22 hepatitis 9.5
23 hepatitis b 9.5
24 vagina, absence of 9.5 MDM2 SMARCB1
25 cranioectodermal dysplasia 1 8.6 BCOR CREBBP MDM2
26 primary eye hypotony 7.7 BCOR CASZ1 CREBBP MDM2 PLIN1 SMARCB1

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:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.72 BCOR CASZ1 CREBBP MDM2 SMARCB1
2 embryo MP:0005380 9.56 BCOR CREBBP MDM2 SMARCB1
3 growth/size/body region MP:0005378 9.55 BCOR CREBBP MDM2 PLIN1 SMARCB1
4 liver/biliary system MP:0005370 9.26 CREBBP MDM2 PLIN1 SMARCB1
5 muscle MP:0005369 9.02 MDM2 PLIN1 SMARCB1 CASZ1 CREBBP

Drugs & Therapeutics for Retroperitoneal Sarcoma

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

(show all 18)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 1, Phase 2 23214-92-8 31703
2
Ifosfamide Approved Phase 2 3778-73-2 3690
3
Cisplatin Approved Phase 2,Phase 1 15663-27-1 84093 441203 2767
4
Trabectedin Approved, Investigational Phase 2 114899-77-3 108150
5
Everolimus Approved Phase 2 159351-69-6 6442177
6
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
7
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
8
Doxil Approved June 1999 Phase 1, Phase 2 31703
9 Anti-Bacterial Agents Phase 1, Phase 2
10 Antibiotics, Antitubercular Phase 1, Phase 2
11 Topoisomerase Inhibitors Phase 1, Phase 2
12 Isophosphamide mustard Phase 2
13 Alkylating Agents Phase 2
14 Antifungal Agents Phase 2
15 Anti-Infective Agents Phase 2
16 Immunosuppressive Agents Phase 2
17 Fluorodeoxyglucose F18
18
Cobalt 7440-48-4 104729

Interventional clinical trials:

(show all 23)

id 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 Pazopanib Maintenance Phase II Unknown status NCT02207309 Phase 2 Pazopanib;Placebo (for Pazopanib)
6 Proton or Photon RT for Retroperitoneal Sarcomas Recruiting NCT01659203 Phase 1, Phase 2
7 Efficacy Study on Trabectedin in Retroperitoneal Leiomyosarcoma and Well Differentiated/Dedifferentiated Liposarcoma Recruiting NCT02247544 Phase 2 Trabectedin
8 Trial of Neoadjuvant Intensity-Modulated Radiation Therapy Followed by Surgery and Intraoperative Radiation Therapy in Resectable Retroperitoneal Soft Tissue Sarcoma (RETRO-WTS) Recruiting NCT01566123 Phase 1, Phase 2
9 Hyperthermia and Proton Therapy in Unresectable Soft Tissue Sarcoma Recruiting NCT01904565 Phase 1, Phase 2
10 High Precision RT For Soft-Tissue Sarcoma Active, not recruiting NCT01389050 Phase 2
11 Assessment of Helical Tomotherapy Radiotherapy (54 Gy) Followed by Surgery in Retro-peritoneal Liposarcoma Active, not recruiting NCT01841047 Phase 2
12 Pilot Study of Hyperthermic Peritoneal Perfusion (HIPEC) for Adolescent and Young Adults With Desmoplastic Small Round Cell Tumor Active, not recruiting NCT01277744 Phase 2 Cisplatin
13 Study of RAD001 in Soft Tissue Extremity and/or Retroperitoneal Sarcomas Terminated NCT01048723 Phase 2 RAD001
14 Combination Chemotherapy, Radiation Therapy, and Surgery in Treating Patients With Primary or Recurrent Sarcoma Withdrawn NCT00017160 Phase 2 doxorubicin;filgrastim;ifosfamide
15 Radiation Therapy Plus Doxorubicin in Treating Patients With Resectable Primary or Recurrent Retroperitoneal Soft Tissue Sarcoma Completed NCT00004123 Phase 1 Doxorubicin hydrochloride (DOX)
16 Continuous Hyperthermic Peritoneal Perfusion (CHPP) With Cisplatin for Children With Peritoneal Cancer Completed NCT00436657 Phase 1 CHPP of Cisplatin
17 Proton Beam Radiation Therapy in Treating Patients With Retroperitoneal Sarcoma Active, not recruiting NCT01034566 Phase 1
18 Contribution of F18-FDG PET/CT to the Early Assessment of Pazopanib Therapy Efficacy in Advanced Soft Tissue Sarcoma Unknown status NCT01594203
19 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
20 Novel Biochemical and Molecular Determinants for Soft Tissue Sarcoma Recruiting NCT00579566
21 Preoperative Imaging in Retroperitoneal Sarcoma Active, not recruiting NCT01902667
22 Pilot Study: Dynamic Contrast-enhanced Magnetic Resonance Imaging(DCE-MRI)for Assessing Tumor Vascularity and Permeability in Soft Tissue Sarcoma Treated With Preoperative Radiotherapy Followed by Surgical Resection Active, not recruiting NCT01575951
23 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

MalaCards organs/tissues related to Retroperitoneal Sarcoma:

39
Kidney, Liver, Bone

Publications for Retroperitoneal Sarcoma

Articles related to Retroperitoneal Sarcoma:

(show top 50) (show all 182)
id Title Authors Year
1
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
2
Predictors of surgical quality for retroperitoneal sarcoma: Volume matters. ( 28608360 )
2017
3
Incidence and Adverse Prognostic Implications of Histopathologic Organ Invasion in Primary Retroperitoneal Sarcoma. ( 28147251 )
2017
4
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
5
Retroperitoneal Sarcoma: Setting the Stage for Treatment Strategies Tailored to Histologic Subtype and Other Patient and Tumor Factors. ( 28871972 )
2017
6
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
7
Post-relapse outcomes after primary extended resection of retroperitoneal sarcoma: A report from the Trans-Atlantic RPS Working Group. ( 28152173 )
2017
8
Needle tract seeding following core biopsies in retroperitoneal sarcoma. ( 28754227 )
2017
9
The modified frailty index to predict morbidity and mortality for retroperitoneal sarcoma resections. ( 28587892 )
2017
10
Importance of preoperative diagnosis for management of patients with suspected retroperitoneal sarcoma. ( 28768365 )
2017
11
Effect of intraoperative radiotherapy in the treatment of retroperitoneal sarcoma. ( 28074298 )
2017
12
Treatment of retroperitoneal sarcoma: current standards and new developments. ( 28509807 )
2017
13
Postoperative Morbidity After Radical Resection of Primary Retroperitoneal Sarcoma: A Report From the Transatlantic RPS Working Group. ( 28394870 )
2017
14
Clinical outcomes of tissue expanders on adjuvant radiotherapy of resected retroperitoneal sarcoma. ( 27755478 )
2016
15
Retroperitoneal Sarcoma: Fact, Opinion, and Controversy. ( 27591493 )
2016
16
Hepatitis B Reactivation After Ifosfamide Therapy for Retroperitoneal Sarcoma. ( 27246626 )
2016
17
Neoadjuvant radiotherapy for retroperitoneal sarcoma: A systematic review. ( 26990903 )
2016
18
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
19
Defining the role of radiotherapy for retroperitoneal sarcoma. ( 27210905 )
2016
20
Spilled gallstones mimicking a retroperitoneal sarcoma following laparoscopic cholecystectomy. ( 27158213 )
2016
21
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
22
Clinical outcomes of tissue expanders on adjuvant radiotherapy of resected retroperitoneal sarcoma. ( 27428199 )
2016
23
Is long-term survival possible after margin-positive resection of retroperitoneal sarcoma (RPS)? ( 27060344 )
2016
24
The Trans-Atlantic Retroperitoneal Sarcoma Working Group (TARPSWG): "Red wine or white"? ( 27613555 )
2016
25
Guidelines for the Treatment of Recurrent Retroperitoneal Sarcoma: Are we Trying to Fit a Square Peg into a Round Hole? ( 27334225 )
2016
26
Predictors of improved survival for patients with retroperitoneal sarcoma. ( 27495850 )
2016
27
The importance of surgical margins in retroperitoneal sarcoma. ( 26707028 )
2016
28
External validation of a multi-institutional retroperitoneal sarcoma nomogram. ( 26916507 )
2016
29
Surgical outcome and patterns of recurrence for retroperitoneal sarcoma at a single centre. ( 26876538 )
2016
30
Practical Issues for Retroperitoneal Sarcoma. ( 27556665 )
2016
31
Management of Recurrent Retroperitoneal Sarcoma (RPS) in the Adult: A Consensus Approach from the Trans-Atlantic RPS Working Group. ( 27480354 )
2016
32
Results of Resection for Recurrent or Residual Retroperitoneal Sarcoma After Failed Primary Treatment. ( 27554502 )
2016
33
CT-guided (125)I seed implantation for inoperable retroperitoneal sarcoma: A technique for delivery of local tumor brachytherapy. ( 28101168 )
2016
34
Giant Retroperitoneal Sarcoma. ( 27613734 )
2016
35
Histology-Based Classification Predicts Pattern of Recurrence and Improves Risk Stratification in Primary Retroperitoneal Sarcoma. ( 25915910 )
2015
36
Comparison of Perioperative Radiation Therapy and Surgery Versus Surgery Alone in 204 Patients With Primary Retroperitoneal Sarcoma: A Retrospective 2-Institution Study. ( 26061213 )
2015
37
Retroperitoneal Sarcoma (RPS) High Risk Gross Tumor Volume Boost (HR GTV Boost) Contour Delineation Agreement Among NRG Sarcoma Radiation and Surgical Oncologists. ( 26018727 )
2015
38
Giant exophytic renal angiomyolipoma mimicking as retroperitoneal sarcoma; a case report with review of literature. ( 26023631 )
2015
39
Analysis of perioperative radiation therapy in the surgical treatment of primary and recurrent retroperitoneal sarcoma. ( 26238282 )
2015
40
Retroperitoneal Sarcoma. Outcome Analysis in a Teaching Hospital in Eastern India- a Perspective. ( 26405413 )
2015
41
Long-term morbidity after multivisceral resection for retroperitoneal sarcoma. ( 26041724 )
2015
42
Retroperitoneal Sarcoma Target Volume and Organ at Risk Contour Delineation Agreement Among NRG Sarcoma Radiation Oncologists. ( 26194680 )
2015
43
Post-operative renal function following nephrectomy as part of en bloc resection of retroperitoneal sarcoma (RPS). ( 26179132 )
2015
44
Treatment Guidelines for Preoperative Radiation Therapy for Retroperitoneal Sarcoma: Preliminary Consensus of an International Expert Panel. ( 26068493 )
2015
45
Impact of chemotherapy on survival in surgically resected retroperitoneal sarcoma. ( 26251340 )
2015
46
Outcome following resection of retroperitoneal sarcoma. ( 26395577 )
2015
47
Acute gastrointestinal toxicity and bowel bag dose-volume parameters for preoperative radiation therapy for retroperitoneal sarcoma. ( 27009922 )
2015
48
Management of recurrent retroperitoneal sarcoma. ( 24155163 )
2014
49
Radiation therapy for retroperitoneal sarcoma. ( 24638910 )
2014
50
Extended Surgical Resection and Histology in Retroperitoneal Sarcoma. ( 25316490 )
2014

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:

id 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 8.32 MDM2

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

id 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
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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 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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