General Information of Disease (ID: DISSN8XB)

Disease Name Soft tissue sarcoma
Synonyms
malignant soft tissue tumour; malignant soft tissue tumor; non-Rhabdo. soft tissue sarcoma; connective tissue sarcoma; soft tissue sarcoma; malignant mesenchymal tumor; sarcoma of the soft tissue; soft part sarcoma; sarcoma of soft tissue; malignant mesenchymal tumour
Disease Class 2B57: Kaposi sarcoma
Definition A malignant neoplasm arising from muscle tissue, adipose tissue, blood vessels, fibrous tissue, or other supportive tissues excluding the bones.
Disease Hierarchy
DISTC6NO: Malignant soft tissue neoplasm
DISZDG3U: Sarcoma
DISSN8XB: Soft tissue sarcoma
ICD Code
ICD-11
ICD-11: 2B57
ICD-9
ICD-9: 171
Expand ICD-9
171
Disease Identifiers
MONDO ID
MONDO_0018078
UMLS CUI
C4551687
MedGen ID
1642116
HPO ID
HP:0030448
Orphanet ID
3394
SNOMED CT ID
424952003

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)
This Disease is Treated as An Indication in 1 Approved Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Olaratumab DMNYOIX Approved Monoclonal antibody [1]
------------------------------------------------------------------------------------
This Disease is Treated as An Indication in 14 Clinical Trial Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Aldoxorubicin DMHKCIS Phase 3 NA [2]
L19-TNF DMPE9AE Phase 3 Fusion protein [3]
TRC105 DM1874J Phase 3 Monoclonal antibody [4]
8H9 I-131 mab DMKADUF Phase 2 Monoclonal antibody [5]
Afamitresgene autoleucel DM1GR4M Phase 2 CAR-T cell therapy [6]
Camsirubicin DMEZLFT Phase 2 Small molecular drug [7]
CMB305 DMYYEM5 Phase 2 NA [8]
GPX-150D DMHNIGB Phase 2 NA [4]
Mecbotamab vedotin DMU5KH4 Phase 2 Antibody drug conjugate [9]
MNPR-201 DMTCSG9 Phase 2 NA [2]
Ontuxizumab DM0FJIE Phase 2 Antibody [4]
Palifosfamide DMMIYZ7 Phase 2 Small molecular drug [10]
TH-302 DMFU0JY Phase 2 Small molecular drug [11]
NC-6300 DM8WXJX Phase 1 NA [2]
------------------------------------------------------------------------------------
⏷ Show the Full List of 14 Drug(s)

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 16 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
DEPDC1 TT8S9CM Limited Biomarker [12]
ANTXR2 TTOD34I moderate Altered Expression [13]
CSPG4 TT7MYXI moderate Biomarker [14]
ENPP2 TTSCIM2 moderate Altered Expression [15]
RGS6 TTJ96M8 moderate Genetic Variation [16]
CD6 TTMF6KC Strong Biomarker [17]
DHCR24 TTTK0NH Strong Biomarker [18]
FGFR4 TT1KX2S Strong Genetic Variation [19]
FUS TTKGYZ9 Strong Genetic Variation [20]
HBEGF TT15SL0 Strong Genetic Variation [21]
LATS2 TTML7FG Strong Genetic Variation [22]
MAGEA3 TTWSKHD Strong Biomarker [23]
MDM2 TT9TE0O Strong Genetic Variation [24]
MTAP TTDBX7N Strong Genetic Variation [25]
PDGFRA TT8FYO9 Strong Biomarker [26]
TACC3 TTQ4UFD Strong Altered Expression [27]
------------------------------------------------------------------------------------
⏷ Show the Full List of 16 DTT(s)
This Disease Is Related to 44 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
CCNB3 OT5V72D3 Limited Biomarker [28]
DEPDC1B OTMVFOT1 Limited Biomarker [12]
PLAGL1 OTZAO900 Limited Posttranslational Modification [29]
ASPSCR1 OTXTOD10 moderate Altered Expression [30]
CAVIN2 OTFHHDRU moderate Biomarker [31]
CD109 OTDADBM4 moderate Altered Expression [32]
CHAMP1 OTBGWU86 moderate Genetic Variation [33]
CIB2 OT9ZJX1I moderate Genetic Variation [34]
CLEC11A OT9KBH7C moderate Altered Expression [35]
CORO7 OTG7MEAJ moderate Biomarker [36]
CREB3L1 OT2JHIHM moderate Genetic Variation [37]
CREB3L2 OT09MHV0 moderate Genetic Variation [37]
IMPACT OTQ923OB moderate Biomarker [38]
ING2 OT6H0EWF moderate Biomarker [39]
KLLN OTV3FPH0 moderate Genetic Variation [40]
MAPK8IP2 OTDUHLN0 moderate Biomarker [41]
MOV10L1 OTP978LK moderate Genetic Variation [33]
MYCL OT1MFQ5U moderate Genetic Variation [42]
PIWIL2 OT1PXQIF moderate Biomarker [43]
PRB2 OTAD4JZ0 moderate Altered Expression [44]
PRDM10 OTDDWU5Q moderate Genetic Variation [45]
PRIMA1 OT9ITT3P moderate Biomarker [46]
SMARCB1 OT2LP7LJ moderate Biomarker [47]
SSX1 OTZ7NRCY moderate Genetic Variation [48]
STK3 OTLNSCQD moderate Posttranslational Modification [49]
TFE3 OTM99ZWH moderate Altered Expression [30]
ANKRD36B OT3MW415 Strong Biomarker [50]
ATF1 OT251CI0 Strong Biomarker [51]
BCOR OTG013AX Strong Genetic Variation [20]
BRINP1 OTEUVSCP Strong Altered Expression [52]
CCAR2 OTLUDG5T Strong Biomarker [52]
CD151 OTF3UZS7 Strong Biomarker [53]
CHSY1 OTB1XSSF Strong Altered Expression [54]
CIC OTFXCHNZ Strong Genetic Variation [20]
EWSR1 OT7SRHV3 Strong Biomarker [20]
EXTL3 OT2BRUBN Strong Altered Expression [54]
IRX2 OTBHXXQ2 Strong Biomarker [55]
NKD2 OTCYT3I6 Strong Altered Expression [55]
PPP2R5E OT8GPFT5 Strong Genetic Variation [56]
S100A1 OT1F2G4J Strong Genetic Variation [24]
SS18 OTDKHN1E Strong Biomarker [57]
SSX2 OT2Z6RLL Strong Biomarker [58]
STOM OTC8R6EH Strong Biomarker [59]
TBC1D9 OTSSCTB5 Strong Altered Expression [60]
------------------------------------------------------------------------------------
⏷ Show the Full List of 44 DOT(s)

References

1 2016 FDA drug approvals. Nat Rev Drug Discov. 2017 Feb 2;16(2):73-76.
2 Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
3 ClinicalTrials.gov (NCT04650984) A Phase III Study Comparing the Efficacy of the Combination of Doxorubicin and the Tumor-targeting Human Antibody-cytokine Fusion Protein L19TNF to Doxorubicin Alone as First-line Therapy in Patients With Advanced or Metastatic Soft Tissue Sarcoma. U.S.National Institutes of Health.
4 Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
5 ClinicalTrials.gov (NCT00582608) Tumor Detection Using Iodine-131-Labeled Monoclonal Antibody 8H9. U.S. National Institutes of Health.
6 ClinicalTrials.gov (NCT04044768) A Phase 2 Single Arm Open-Label Clinical Trial of ADP-A2M4 SPEAR? T Cells in Subjects With Advanced Synovial Sarcoma or Myxoid/Round Cell Liposarcoma. U.S.National Institutes of Health.
7 Clinical pipeline report, company report or official report of Monopar Therapeutics.
8 Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
9 Clinical pipeline report, company report or official report of BioAtla
10 ClinicalTrials.gov (NCT01808534) Palifosfamide in Treating Patients With Recurrent Germ Cell Tumors. U.S. National Institutes of Health.
11 URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 8695).
12 Prognostic role of XTP1/DEPDC1B and SDP35/DEPDC1A in high grade soft-tissue sarcomas.Histol Histopathol. 2018 Jun;33(6):597-608. doi: 10.14670/HH-11-959. Epub 2018 Jan 3.
13 CMG2 Expression Is an Independent Prognostic Factor for Soft Tissue Sarcoma Patients.Int J Mol Sci. 2017 Dec 7;18(12):2648. doi: 10.3390/ijms18122648.
14 Effects of chondroitin sulfate proteoglycan 4 (NG2/CSPG4) on soft-tissue sarcoma growth depend on tumor developmental stage.J Biol Chem. 2018 Feb 16;293(7):2466-2475. doi: 10.1074/jbc.M117.805051. Epub 2017 Dec 1.
15 c-Jun promotes cell migration and drives expression of the motility factor ENPP2 in soft tissue sarcomas.J Pathol. 2014 Oct;234(2):190-202. doi: 10.1002/path.4379. Epub 2014 Aug 1.
16 Absence of mutation at the GAP-related domain of the neurofibromatosis type 1 gene in sporadic neurofibrosarcomas and other bone and soft tissue sarcomas.Cancer Genet Cytogenet. 1995 Jun;81(2):173-4. doi: 10.1016/0165-4608(94)00267-f.
17 No rearrangements of the CHOP gene in malignant fibrous histiocytoma.Cancer Genet Cytogenet. 1994 Feb;72(2):155-6. doi: 10.1016/0165-4608(94)90133-3.
18 Soft tissue sarcoma: DWI and DCE-MRI parameters correlate with Ki-67 labeling index.Eur Radiol. 2020 Feb;30(2):914-924. doi: 10.1007/s00330-019-06445-9. Epub 2019 Oct 18.
19 The fibroblast growth factor receptor 4 (FGFR4) Arg388 allele correlates with survival in head and neck squamous cell carcinoma.Exp Mol Pathol. 2007 Feb;82(1):53-7. doi: 10.1016/j.yexmp.2006.05.003. Epub 2006 Nov 3.
20 DNA methylation profiling distinguishes Ewing-like sarcoma with EWSR1-NFATc2 fusion from Ewing sarcoma.J Cancer Res Clin Oncol. 2019 May;145(5):1273-1281. doi: 10.1007/s00432-019-02895-2. Epub 2019 Mar 20.
21 First-in-man phase I study assessing the safety and pharmacokinetics of a 1-hour intravenous infusion of the doxorubicin prodrug DTS-201 every 3 weeks in patients with advanced or metastatic solid tumours.Eur J Cancer. 2017 Nov;86:240-247. doi: 10.1016/j.ejca.2017.09.009. Epub 2017 Oct 19.
22 LATS tumor suppressor: a new governor of cellular homeostasis.Cell Cycle. 2010 Oct 1;9(19):3892-903. doi: 10.4161/cc.9.19.13386. Epub 2010 Oct 20.
23 MAGE-A3 is a Clinically Relevant Target in Undifferentiated Pleomorphic Sarcoma/Myxofibrosarcoma.Cancers (Basel). 2019 May 15;11(5):677. doi: 10.3390/cancers11050677.
24 GLI1-amplifications expand the spectrum of soft tissue neoplasms defined by GLI1 gene fusions.Mod Pathol. 2019 Nov;32(11):1617-1626. doi: 10.1038/s41379-019-0293-x. Epub 2019 Jun 12.
25 Identification of a novel MTAP-RAF1 fusion in a soft tissue sarcoma.Diagn Pathol. 2018 Oct 12;13(1):77. doi: 10.1186/s13000-018-0759-z.
26 Olaratumab combined with doxorubicin and ifosfamide overcomes individual doxorubicin and olaratumab resistance of an undifferentiated soft-tissue sarcoma in a PDOX mouse model.Cancer Lett. 2019 Jun 1;451:122-127. doi: 10.1016/j.canlet.2019.03.003. Epub 2019 Mar 10.
27 Elevated Expression of Transforming Acidic Coiled-Coil Containing Protein 3 (TACC3) Is Associated With a Poor Prognosis in Osteosarcoma.Clin Orthop Relat Res. 2018 Sep;476(9):1848-1855. doi: 10.1097/CORR.0000000000000379.
28 BCOR-CCNB3-positive soft tissue sarcoma with round-cell and spindle-cell histology: a series of four cases highlighting the pitfall of mimicking poorly differentiated synovial sarcoma.Histopathology. 2016 Nov;69(5):792-801. doi: 10.1111/his.13001. Epub 2016 Jul 15.
29 Prognostic value of PLAGL1-specific CpG site methylation in soft-tissue sarcomas.PLoS One. 2013 Nov 15;8(11):e80741. doi: 10.1371/journal.pone.0080741. eCollection 2013.
30 Expression of MET in alveolar soft part sarcoma.Med Oncol. 2010 Jun;27(2):459-65. doi: 10.1007/s12032-009-9234-8. Epub 2009 May 27.
31 Cavin-2 is a specific marker for detection of well-differentiated liposarcoma.Biochem Biophys Res Commun. 2017 Nov 4;493(1):660-665. doi: 10.1016/j.bbrc.2017.08.135. Epub 2017 Sep 1.
32 High expression of CD109 antigen regulates the phenotype of cancer stem-like cells/cancer-initiating cells in the novel epithelioid sarcoma cell line ESX and is related to poor prognosis of soft tissue sarcoma.PLoS One. 2013 Dec 20;8(12):e84187. doi: 10.1371/journal.pone.0084187. eCollection 2013.
33 Cytogenetic analysis of 46 pleomorphic soft tissue sarcomas and correlation with morphologic and clinical features: a report of the CHAMP Study Group. Chromosomes and MorPhology.Genes Chromosomes Cancer. 1998 May;22(1):16-25. doi: 10.1002/(sici)1098-2264(199805)22:1<16::aid-gcc3>3.0.co;2-a.
34 Cyclin-dependent kinase inhibitor p57KIP2 in soft tissue sarcomas and Wilms'tumors.Cancer Res. 1996 Mar 15;56(6):1219-21.
35 Clinical Benefit of Pazopanib in a Patient with Metastatic Chondrosarcoma: A Case Report and Review of the Literature.Front Oncol. 2018 Mar 1;8:45. doi: 10.3389/fonc.2018.00045. eCollection 2018.
36 Postoperative Day 1 Glucose May Be Associated With Wound Complications in Sarcomas Treated With Preoperative Radiation.Clin Orthop Relat Res. 2018 Mar;476(3):580-586. doi: 10.1007/s11999.0000000000000056.
37 Low-grade fibromyxoid sarcoma with prominent giant rosettes and heterotopic ossification.Pathol Res Pract. 2012 Sep 15;208(9):557-60. doi: 10.1016/j.prp.2012.06.002. Epub 2012 Jul 20.
38 ROS1-GOPC/FIG: a novel gene fusion in hepatic angiosarcoma.Oncotarget. 2019 Jan 4;10(2):245-251. doi: 10.18632/oncotarget.26521. eCollection 2019 Jan 4.
39 Targeted disruption of Ing2 results in defective spermatogenesis and development of soft-tissue sarcomas.PLoS One. 2010 Nov 19;5(11):e15541. doi: 10.1371/journal.pone.0015541.
40 Alveolar rhabdomyosarcoma in an adolescent male patient - case report and current perspectives.Rom J Morphol Embryol. 2018;59(4):1247-1252.
41 Investigating a chimeric anti-mouse PDGFR antibody as a radiosensitizer in primary mouse sarcomas.EBioMedicine. 2019 Feb;40:224-230. doi: 10.1016/j.ebiom.2019.01.046. Epub 2019 Jan 31.
42 Elevated frequency of a specific allele of the L-myc gene in male patients with bone and soft-tissue sarcomas.Int J Cancer. 1990 Jan 15;45(1):47-9. doi: 10.1002/ijc.2910450110.
43 Expression of human Piwi-like genes is associated with prognosis for soft tissue sarcoma patients.BMC Cancer. 2012 Jun 29;12:272. doi: 10.1186/1471-2407-12-272.
44 The retinoblastoma family member pRb2/p130 is an independent predictor of survival in human soft tissue sarcomas.Clin Cancer Res. 2008 Aug 1;14(15):4775-9. doi: 10.1158/1078-0432.CCR-07-4055.
45 Recurrent PRDM10 gene fusions in undifferentiated pleomorphic sarcoma.Clin Cancer Res. 2015 Feb 15;21(4):864-9. doi: 10.1158/1078-0432.CCR-14-2399. Epub 2014 Dec 16.
46 PRIMA-1(MET) induces death in soft-tissue sarcomas cell independent of p53.BMC Cancer. 2015 Oct 13;15:684. doi: 10.1186/s12885-015-1667-1.
47 The oncomir face of microRNA-206: A permanent miR-206 transfection study.Exp Biol Med (Maywood). 2018 Aug;243(12):1014-1023. doi: 10.1177/1535370218795406. Epub 2018 Aug 15.
48 Real-time polymerase chain reaction as an aid for the detection of SYT-SSX1 and SYT-SSX2 transcripts in fresh and archival pediatric synovial sarcoma specimens: report of 25 cases from St. Jude Children's Research Hospital.Pediatr Dev Pathol. 2003 Jan-Feb;6(1):24-34. doi: 10.1007/s10024-002-0050-9. Epub 2002 Dec 10.
49 Frequent hypermethylation of MST1 and MST2 in soft tissue sarcoma.Mol Carcinog. 2007 Oct;46(10):865-71. doi: 10.1002/mc.20317.
50 Immunohistochemical expression and clinicopathological assessment of the cancer testis antigens NY-ESO-1 and MAGE-A4 in high-grade soft-tissue sarcoma.Oncol Lett. 2019 Apr;17(4):3937-3943. doi: 10.3892/ol.2019.10044. Epub 2019 Feb 14.
51 Fusion of the EWSR1 and ATF1 genes without expression of the MITF-M transcript in angiomatoid fibrous histiocytoma.Genes Chromosomes Cancer. 2005 Sep;44(1):97-102. doi: 10.1002/gcc.20201.
52 Expression of SIRT1 and DBC1 is associated with poor prognosis of soft tissue sarcomas.PLoS One. 2013 Sep 3;8(9):e74738. doi: 10.1371/journal.pone.0074738. eCollection 2013.
53 CD151 confers metastatic potential to clear cell sarcoma of the soft tissue in animal model.Oncol Lett. 2019 Jun;17(6):4811-4818. doi: 10.3892/ol.2019.10164. Epub 2019 Mar 19.
54 Chondroitin sulfate synthase 1 expression is associated with malignant potential of soft tissue sarcomas with myxoid substance.Hum Pathol. 2016 Apr;50:15-23. doi: 10.1016/j.humpath.2015.11.005. Epub 2015 Nov 22.
55 Frequent amplifications and abundant expression of TRIO, NKD2, and IRX2 in soft tissue sarcomas.Genes Chromosomes Cancer. 2006 Sep;45(9):829-38. doi: 10.1002/gcc.20343.
56 PP2A:B56{epsilon}, a substrate of caspase-3, regulates p53-dependent and p53-independent apoptosis during development.J Biol Chem. 2010 Nov 5;285(45):34493-502. doi: 10.1074/jbc.M110.169581. Epub 2010 Aug 31.
57 The prognostic impact of SYT-SSX fusion type and histological grade in pediatric patients with synovial sarcoma treated according to the CWS (Cooperative Weichteilsarkom Studie) trials.Pediatr Blood Cancer. 2017 Jan;64(1):89-95. doi: 10.1002/pbc.26206. Epub 2016 Sep 13.
58 A novel sarcoma with dual differentiation: clinicopathologic and molecular characterization of a combined synovial sarcoma and extraskeletal myxoid chondrosarcoma.Am J Surg Pathol. 2012 Jul;36(7):1093-8. doi: 10.1097/PAS.0b013e31824cd174.
59 Simultaneous expression of flotillin-1, flotillin-2, stomatin and caveolin-1 in non-small cell lung cancer and soft tissue sarcomas.BMC Cancer. 2014 Feb 17;14:100. doi: 10.1186/1471-2407-14-100.
60 MDR1 gene expression: evaluation of its use as a molecular marker for prognosis and chemotherapy of bone and soft tissue sarcomas.Eur J Cancer. 1996 Jan;32A(1):86-92. doi: 10.1016/0959-8049(95)00478-5.