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Review

A review of soft-tissue sarcomas: translation of biological advances into treatment measures

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Pages 1089-1114 | Published online: 10 May 2018

References

  • JemalATiwariRCMurrayTCancer statistics, 2004CA Cancer J Clin20045482914974761
  • SiegelRLMillerKDJemalACancer statisticsCA Cancer J Clin20156552925559415
  • LawrenceWJrDoneganWLNtarajanNMettlinCBeartRWinchesterDAdult soft tissue sarcomas: a pattern of care survey of the American College of SurgeonsAnn Surg19872053493593566372
  • Christie-LargeMJamesSLTiessenLDaviesAMGrimerRJImaging strategy for detecting lung metastases at presentation in patients with soft tissue sarcomasEur J Cancer2008441841184518640829
  • American Cancer SocietySurvival by stage of soft tissue sarcoma2017 Available from: https://www.cancer.org/cancer/soft-tissue-sarcoma/detection-diagnosis-staging/survival-rates.htmlAccessed March 28, 2018
  • EdgeSBByrdDRComptonCCFritzAGGreeneFLTrottiACancer Staging Manual7th edHeidelbergSpringer2010
  • ZagarsGKBalloMTPistersPWPrognostic factors for patients with localized soft-tissue sarcoma treated with conservation surgery and radiation therapy: an analysis of 1225 patientsCancer2003972530254312733153
  • FletcherCDBridgeJAHogendoornPCMertensFWHO Classification of Tumours of Soft Tissue and Bone4th edGenevaWorld Health Organization2013
  • KurodaMIshidaTHoriuchiHChimeric TLS/FUS-CHOP gene expression and the heterogeneity of its junction in human myxoid and round cell liposarcomaAm J Pathol1995147122112277485386
  • CragoASocciNDDeCarolisPCopy number losses define subgroups of dedifferentiated liposarcoma with poor prognosis and genomic instabilityClin Cancer Res20021813341340
  • BellTWhat is liposarcoma?2012 Available from: http://sarcomahelp.org/liposarcoma.htmlAccessed March 28, 2018
  • CragoAMSingerSClinical and molecular approaches to well-differentiated and dedifferentiated liposarcomaCurr Opin Oncol20112337337821552124
  • KanojiaDNagataYGargMGenomic landscape of liposarcomaOncotarget20156424294244426643872
  • RiekerRJWeitzLehnerBGenomic profiling reveals subsets of dedifferentiated liposarcoma to follow separate molecular pathwaysVirchows Arch201045627728520039060
  • AntonescuCRTschernyavskySJDecusearaRPrognostic impact of P53 status, TLS-CHOP fusion transcript structure, and histological grade in myxoid liposarcoma: a molecular and clinicopathologic study of 82 casesClin Cancer Res200173977398711751490
  • RiekerRJJoosSBartschCDistinct chromosomal imbalances in pleomorphic and in high-grade dedifferentiated liposarcomasInt J Cancer200299687311948494
  • Spinella-JaegleSRawadiGKawaiSSonic hedgehog increases the commitment of pluripotent mesenchymal cells into osteoblastic lineage and abolishes adipocytic differentiationJ Cell Sci20011142085209411493644
  • GobbleRMQinLXBrillERExpression profiling of liposarcoma yields a multigene predictor of patient outcome and identifies genes that contribute to liposarcomagenesisCancer Res2011712697270521335544
  • CostaADaidoneMGDapraiLTelomere maintenance mechanisms in liposarcomas: association with histologic subtypes and disease progressionCancer Res2006668918892416951210
  • ItalianoABianchiniLGjernesEClinical and biological significance of CDK4 amplification in well-differentiated and dedifferentiated liposarcomasClin Cancer Res2009155696570319737942
  • TapWDEilberFCGintherCEvaluation of well-differentiated/de-differentiated liposarcomas by high-resolution oligonucleotide array-based comparative genomic hybridizationGenes Chromosomes Cancer2011509511221117066
  • TaylorBSDeCarolisPLAngelesCVFrequent alterations and epigenetic silencing of differentiation pathway genes in structurally rearranged liposarcomasCancer Discov2011158759722328974
  • SingerSSocciNDAmbrosiniGGene expression profiling of liposarcoma identifies distinct biological types/subtypes and potential therapeutic targets in well-differentiated and dedifferentiated liposar-comaCancer Res2007676626663617638873
  • CoindreJMPédeutourFAuriasAWell-differentiated and dedifferentiated liposarcomasVirchows Arch201045616717919688222
  • SchmidtHBartelFKapplerMGains of 13q are correlated with a poor prognosis in liposarcomaMod Pathol20051863864415540119
  • KoelscheCRennerMHartmannWTERT promoter hotspot mutations are recurrent in myxoid liposarcomas but rare in other soft tissue sarcoma entitiesJ Exp Clin Cancer Res2014333324726063
  • SreekantaiahCThe cytogenetic and molecular characterization of benign and malignant soft tissue tumorsCytogenet Cell Genet19988213299763652
  • FritzBSchubertFWrobelGMicroarray-based copy number and expression profiling in dedifferentiated and pleomorphic liposarcomaCancer Res2002622993299812036902
  • SchoffskiPRay-CoquardILCioffiAActivity of eribulin mesylate in patients with soft-tissue sarcoma: a phase 2 study in four independent histological subtypesLancet2011121045105221937277
  • SchoffskiPChawlaSMakiRGEribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicenter, phase 3 trialLancet20163871629163726874885
  • GrossoFJonesRLDemetriGDEfficacy of trabectedin in advanced pretreated myxoid liposarcomas: a retrospective studyLancet Oncol2007859560217586092
  • GiandomenicoSDFrapolliRBelloEMode of action of trabectedin in myxoid liposarcomaOncogene2014335201521024213580
  • DicksonMATapWDKeohanMLPhase II trial of the CDK4 inhibitor PD0332991 in patients with advanced CDK4-amplified well-differentiated or dedifferentiated liposarcomaJ Clin Oncol2013312024202823569312
  • Memorial Sloan Kettering Cancer CenterStudy of abemaciclib in dedifferentiated liposarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT02846987. NLM identifier: NCT02846987Accessed March 28, 2018
  • Hadassah Medical OrganizationA phase II single arm study assessing efficacy and safety of ribociclib in patients with advanced well-differentiated or dedifferentiated liposarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT02571829. NLM identifier: NCT02571829Accessed March 28, 2018
  • Vector OncologyA phase II study of pazopanib in the treatment of surgically unresectable or metastatic liposarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT01506596. NLM identifier: NCT01506596Accessed March 28, 2018
  • Grupo Espanol de Investigacion en SarcomasPhase II clinical trial of pazopanib to evaluate the activity and tolerability in patients with advanced and/or metastatic liposarcoma who have relapsed following standard therapies or for whom no standard therapy exists Available from: https://clinicaltrials.gov/ct2/show/NCT01692496. NLM identifier: NCT01692496Accessed March 28, 2018
  • Northwestern UniversityA phase II study of pazopanib with oral topotecan in patients with metastatic and non-resectable soft tissue and bone sarcomas Available from: https://clinicaltrials.gov/ct2/show/NCT02357810. NLM identifier: NCT02357810Accessed March 28, 2018
  • ChawlaSPStaddonAPBakerLHPhase II study of the mammalian target of rapamycin inhibitor ridaforolimus in patients with advanced bone and soft tissue sarcomasJ Clin Oncol201230788422067397
  • MD Anderson Cancer CenterStudy of neoadjuvant checkpoint blockade in patients with surgically resectable undifferentiated pleomorphic sarcoma and dedifferentiated liposarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT03307616. NLM identifier: NCT03307616Accessed March 28, 2018
  • National Cancer InstituteRandomized phase II study of nivolumab with or without ipilimumab in patients with metastatic or unresectable sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT02500797. NLM identifier: NCT02500797Accessed March 28, 2018
  • Fox Chase Cancer CenterPhase II trial of ribociclib and everolimus in advanced dedifferentiated liposarcoma (DDL) and leiomyosarcoma (LMS) Available from: https://clinicaltrials.gov/ct2/show/NCT03114527. NLM identifier: NCT03114527Accessed March 28, 2018
  • AdaptimmuneA pilot study of NY-ESO-1c259 T cells in subjects with advanced myxoid/round cell liposarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT02992743. NLM identifier: NCT02992743Accessed March 28, 2018
  • Fred Hutchinson Cancer Research CenterAvelumab and trabectedin in treating patients with liposarcoma or leiomyosarcoma that is metastatic or cannot be removed Available from: https://clinicaltrials.gov/ct2/show/NCT03074318. NLM identifier: NCT03074318Accessed March 28, 2018
  • Immune DesignA randomized, open-label, phase 2 trial of CMB305 (sequentially administered LV305 and G305) and atezolizumab in patients with locally advanced, relapsed, or metastatic sarcoma expressing NY-ESO-1 Available from: https://clinicaltrials.gov/ct2/show/NCT02609984. NLM identifier: NCT02609984Accessed March 28, 2018
  • Sarcoma Oncology Research CenterA phase 1B single center investigation of safety/efficacy of nivolumab (Opdivo) and ABI-009 (nab-rapamycin) in patients with advanced undifferentiated pleomorphic sarcoma, liposarcoma, chondrosarcoma, osteosarcoma and Ewing sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT03190174. NLM identifier: NCT03190174Accessed March 28, 2018
  • Washington University School of MedicineA non-inferiority study of doxorubicin with upfront dexrazoxane plus olaratumab for the treatment of advanced or metastatic soft tissue sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT02584309. NLM identifier: NCT02584309Accessed March 28, 2018
  • LammWNatterCSchurSDistinctive outcome in patients with non-uterine and uterine leiomyosarcomaBMC Cancer20141498125523155
  • YoungRJBrownNJReedMWHughesDWollPJAngiosarcomaLancet Oncol20101198399120537949
  • FayetteJMartinEPiperno-NeumannSAngiosarcomas, a heterogeneous group of sarcomas with specific behavior depending on primary site: a retrospective study of 161 casesAnn Oncol2007182030203617974557
  • StrobbeLJPeterseHLvan TinterenHWijnmaalenARutgersEJAngiosarcoma of the breast after conservation therapy for invasive cancer, the incidence and outcome: an unforseen [sic] sequelaBreast Cancer Res Treat1998471011099497098
  • MuraliRChandramohanRMöllerITargeted massively parallel sequencing of angiosarcomas reveals frequent activation of the mitogen activated protein kinase pathwayOncotarget20156360413605226440310
  • ItakuraEYamamotoHOdaYTsuneyoshiMDetection and characterization of vascular endothelial growth factors and their receptors in a series of angiosarcomasJ Surg Oncol200897748118041747
  • ItalianoAChenCLThomasRAlterations of the p53 and PIK3CA/AKT/mTOR pathways in angiosarcomas: a pattern distinct from other sarcomas with complex genomicsCancer20121185878588722648906
  • MannerJRadlwimmerBHohenbergerPMYC high level gene amplification is a distinctive feature of angiosarcomas after irradiation or chronic lymphedemaAm J Pathol2010176343920008140
  • ShonWSukovWRJenkinsSMFolpeALMYC amplification and overexpression in primary cutaneous angiosarcoma: a fluorescence in-situ hybridization and immunohistochemical studyMod Pathol20142750951524091875
  • LaéMLebelAHamel-ViardFCan c-Myc amplification reliably discriminate postradiation from primary angiosarcoma of the breast?Cancer Radiother20151916817425863565
  • FernandezAPSunYTubbsRRGoldblumJRBillingsSDFISH for MYC amplification and anti-MYC immunohistochemistry: useful diagnostic tools in the assessment of secondary angiosarcoma and atypical vascular proliferationsJ Cutan Pathol20123923424222121953
  • GinterPSMosqueraJMMacDonaldTYD’AlfonsoTMRubinMAShinSJDiagnostic utility of MYC amplification and anti-MYC immunohistochemistry in atypical vascular lesions, primary or radiation-induced mammary angiosarcomas, and primary angiosarcomas of other sitesHum Pathol201445470971624457083
  • GuoTZhangLChangNESingerSMakiRGAntonescuCRConsistent MYC and FLT4 gene amplification in radiation-induced angiosarcoma but not in other radiation-associated atypical vascular lesionsGenes Chromosomes Cancer201150253320949568
  • MentzelTSchildhausHUPalmedoGBüttnerRKutznerHPostradiation cutaneous angiosarcoma after treatment of breast carcinoma is characterized by MYC amplification in contrast to atypical vascular lesions after radiotherapy and control cases: clinicopathological, immunohistochemical and molecular analysis of 66 casesMod Pathol201225758521909081
  • TolaniBGopalakrishnanRPunjVTargeting Myc in KSHV-associated primary effusion lymphoma with BET bromodomain inhibitorsOncogene201429332928293723792448
  • PottiAGantiAKTendulkarKDetermination of vascular endothelial growth factor (VEGF) overexpression in soft tissue sarcomas and the role of overexpression in leiomyosarcomaJ Cancer Res Clin Oncol2004130525614600832
  • PottiAGantiAKFosterHImmunohistochemical detection of HER-2/neu, c-Kit (CD117) and vascular endothelial growth factor (VEGF) overexpression in soft tissue sarcomasAnticancer Res20042433333715015617
  • PakosEEGoussiaACTsekerisPGPapachristouDJStefanouDAgnantisNJExpression of vascular endothelial growth factor and its receptor, KDR/Flk-1, in soft tissue sarcomasAnticancer Res2005253591359616101185
  • ChaoCAl-SaleemTBrooksJJRogatkoAKraybillWGEisenbergBVascular endothelial growth factor and soft tissue sarcomas: tumor expression correlates with gradeAnn Surg Oncol2001826026711314944
  • RanieriGMammìMDi PaolaEEPazopanib a tyrosine kinase inhibitor with strong anti-angiogenetic activity: a new treatment for metastatic soft tissue sarcomaCrit Rev Oncol Hematol20148932232924041629
  • AntonescuCRYoshidaAGuoTKDR activating mutations in human angiosarcomas are sensitive to specific kinase inhibitorsCancer Res2009697175717919723655
  • AmoYMasuzawaMHamadaYKatsuokaKExpression of vascular endothelial growth factor in a human hemangiosarcoma cell line (ISO-HAS)Arch Dermatol Res200129329630111480589
  • BehjatiSTarpeyPSSheldonHRecurrent PTPRB and PLCG1 mutations in angiosarcomaNat Genet20144637637924633157
  • MellbergSDimbergABahramFTranscriptional profiling reveals a critical role for tyrosine phosphatase VE-PTP in regulation of VEGFR2 activity and endothelial cell morphogenesisFASEB J2009231490150219136612
  • RaviVPatelSVascular sarcomasCurr Oncol Rep20131534735523852636
  • von MehrenMRankinCGoldblumJRPhase 2 Southwest Oncology Group-directed intergroup trial (S0505) of sorafenib in advanced soft tissue sarcomasCancer201211877077621751200
  • PenelNRay-CoquardIBal-MahieuCLow level of baseline circulating VEGF-A is associated with better outcome in patients with vascular sarcomas receiving sorafenib: an ancillary study from a phase II trialTarget Oncol2014927327724218035
  • HurwitzHIDowlatiASainiSPhase I trial of pazopanib in patients with advanced cancerClin Cancer Res2009154220422719509175
  • van der GraafWTBlayJYChawlaSPPazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trialLancet201227918791886
  • VerweijJSleijferSPazopanib, a new therapy for metastatic soft tissue sarcomaExpert Opin Pharmacother20131492993523488774
  • OgataDYanagisawaHSuzukiKOashiKYamazakiNTsuchidaTPazopanib treatment slows progression and stabilizes disease in patients with taxane-resistant cutaneous angiosarcomaMed Oncol20163311627613162
  • RosenAThimonSTernantDMachetMCPaintaudGMachetLPartial response to bevacizumab of an extensive cutaneous angiosarcoma of the faceBr J Dermatol201016322522720394623
  • AgulnikMYarberJLOkunoSHAn open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomasAnn Oncol20132425726322910841
  • KoontzBFMilesEFRubioMAPreoperative radiotherapy and bevacizumab for angiosarcoma of the head and neck: two case studiesHead Neck200830262617685450
  • FullerCKCharlsonJADankleSKRussellTJDramatic improvement of inoperable angiosarcoma with combination paclitaxel and bevacizumab chemotherapyJ Am Acad Dermatol201063e83e8420846560
  • DicksonMAD’AdamoDRKeohanMLPhase II trial of gemcitabine and docetaxel with bevacizumab in soft tissue sarcomaSarcoma2015201553247826074722
  • MakiRGJungbluthAAGnjaticSA pilot study of anti-CTLA4 antibody ipilimumab in patients with synovial sarcomaSarcoma2013201316814523554566
  • SindhuSGimberLHCranmerLMcBrideAKraftASAngiosarcoma treated successfully with anti-PD-1 therapy: a case reportJ Immunother Cancer201755828716069
  • LammWNatterCSchurSDistinctive outcome in patients with non-uterine and uterine leiomyosarcomaBMC Cancer20141498125523155
  • GuoXJoVYMillsAMClinically relevant molecular subtypes in leiomyosarcomaClin Cancer Res2015213501251125896974
  • HernandoECharytonowiczEDudasMEThe AKT-mTOR pathway plays a critical role in the development of leiomyosarcomaNat Med20071374875317496901
  • ShiGPerleMAMittalKLet-7 repression leads to HMGA2 overexpression in uterine leiomyosarcomaJ Cell Mol Med2009133898390519602040
  • HayashiTHoriuchiASanoKPotential Role of LMP2 as tumor-suppressor defines new targets for uterine leiomyosarcoma therapySci Rep2011118022355695
  • ShanWAkinfenwaPYSavannahKBA small-molecule inhibitor targeting the mitotic spindle checkpoint impairs the growth of uterine leiomyosarcomaClin Cancer Res2012183352336522535157
  • SetiaAKanotraSAggarwalRBhavthankarDPEpithelioid leiomyosarcoma of uterusBMJ Case Rep2012
  • EdrisBWeiskopfKVolkmerAKAntibody therapy targeting the CD47 protein is effective in a model of aggressive metastatic leiomyosarcomaProc Natl Acad Sci U S A20121096656666122451919
  • HuJRaoUNJasaniSKhannaVYawKSurtiULoss of DNA copy number of 10q is associated with aggressive behavior of leiomyosarcoma: a comparative genomic hybridization studyCancer Genet Cytogenet2005161202716080954
  • HuJKhannaVJonesMSurtiUGenomic alterations in uterine leiomyosarcomas: potential markers for clinical diagnosis and prognosisGenes Chromosomes Cancer20013111712411319799
  • PonceletCWalkerFMedelenatPExpression of CD44 standard and isoforms V3 and V6 in uterine smooth muscle tumors: a possible diagnostic tool for the diagnosis of leiomyosarcomaHum Pathol20093211901196
  • SavannahKJDemiccoEGDual targeting of mTOR and aurora-A kinase for the treatment of uterine leiomyosarcomaClin Cancer Res2012184633464522821997
  • SchvartzmanJMSotilloRBenezraBMitotic chromosomal instability and cancer: mouse modeling of the human diseaseNat Rev Cancer20101010211520094045
  • EdrisBEspinosaIMühlenburgTROR2 is a novel prognostic biomarker and a potential therapeutic target in leiomyosarcoma and gastrointestinal stromal tumourJ Pathol201222722323322294416
  • GiraudetALBadelJNCassierPSYNFRIZZ: a phase IA/IB of a radiolabelled monoclonal AB for the treatment of relapsing synovial sarcomaJ Nucl Med20145522324434294
  • SetteGSalvatiVMemeoLEGFR inhibition abrogates leiomyosarcoma cell chemoresistance through inactivation of survival pathways and impairment of CSC potentialPLoS One2012710e4689123056514
  • HayashiTFaustmanDLDevelopment of spontaneous uterine tumors in low molecular mass polypeptide-2 knockout miceCancer Res200262242711782352
  • MakiRGKraftASScheuKA multicenter phase II study of bortezomib in recurrent or metastatic sarcomasCancer20051031431143815739208
  • GaoCFXieQZhangYWTherapeutic potential of hepatocyte growth factor/scatter factor neutralizing antibodies: inhibition of tumor growth in both autocrine and paracrine hepatocyte growth factor/scatter factor – c-Met-driven models of leiomyosarcomaMol Cancer Ther200982803281019825800
  • BurgessTCoxonAMeyerSFully human monoclonal antibodies to hepatocyte growth factor with therapeutic potential against hepatocyte growth factor/c-Met-dependent human tumorsCancer Res2006661721172916452232
  • IvesonTDonehowerRCDavidenkoIRilotumumab in combination with epirubicin, cisplatin, and capecitabine as first-line treatment for gastric or oesophagogastric junction adenocarcinoma: an open-label, dose de-escalation phase B study and a double-blind, randomized phase 2 studyLancet Oncol2014151007101824965569
  • AmgenA multicenter, phase 1/1B, open label study evaluating the safety, tolerability and pharmacokinetics of rilotumumab in Japanese subjects Available from: https://clinicaltrials.gov/ct2/show/NCT01791374. NLM identifier: NCT01791374Accessed March 28, 2018
  • Gynecologic Oncology GroupA phase II evaluation of AMG 102 (rilotumumab) (NSC #750009) in the treatment of persistent or recurrent epithelial ovarian, fallopian tube, or primary peritoneal carcinoma Available from: https://clinicaltrials.gov/ct2/show/NCT01039207. NLM identifier: NCT01039207Accessed March 28, 2018
  • University of PittsburghA phase I/II trial of AMG 102 and erlotinib in previously treated patients with advanced non-small cell lung cancer Available from: https://clinicaltrials.gov/ct2/show/NCT01233687. NLM identifier: NCT01233687Accessed March 28, 2018
  • Duke University Medical CenterPhase II study to evaluate the efficacy and safety of AMG 102 and Avastin in subjects with recurrent malignant glioma Available from: https://clinicaltrials.gov/ct2/show/NCT01113398. NLM identifier: NCT01113398Accessed March 28, 2018
  • Southwest Oncology GroupA biomarker-driven master protocol for previously treated squamous cell lung cancer (Lung-MAP) Available from: https://clinicaltrials.gov/ct2/show/NCT02154490. NLM identifier: NCT02154490Accessed March 28, 2018
  • PaoluzziLCacavioAGhesaniMResponse to anti-PD1 therapy with nivolumab in metastatic sarcomasClin Sarcoma Res201662428042471
  • Royal Marsden NHS Foundation TrustA phase I study to assess the safety and tolerability of pembrolizumab in combination with fixed rate gemcitabine chemotherapy in patients with leiomyosarcoma and undifferentiated pleomorphic sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT03123276. NLM identifier: NCT03123276Accessed March 28, 2018
  • Institut BergoniéCombination of MK3475 and metronomic cyclophosphamide in patients with advanced sarcomas: multicentre phase II trial Available from: https://clinicaltrials.gov/ct2/show/NCT02406781. NLM identifier: NCT02406781Accessed March 28, 2018
  • HeineAKristiansenGSchildHHBrossartPSuccessful treatment of refractory leiomyosarcoma with PD-1 inhibitor nivolumabAnn Oncol2016271813181427329248
  • GeorgeSMiaoDDemetriGDLoss of PTEN is associated with resistance to anti-PD-1 checkpoint blockade therapy in metastatic uterine leiomyosarcomaImmunity20174619720428228279
  • EdrisBWeiskopfKWeissmanILvan de RijnMFlipping the script on macrophages in leiomyosarcomaOncoimmunology201211202120423170280
  • Forty SevenA phase I dose escalation trial of the humanized anti-CD47 monoclonal antibody Hu5F9-G4 in haematological malignancies (CAMELLIA) Available from: https://clinicaltrials.gov/ct2/show/NCT02678338. NLM identifier: NCT02678338Accessed March 28, 2018
  • CelgeneA phase 1, open-label, dose finding study of CC-90002, a monoclonal antibody directed against CD47, in subjects with acute myeloid leukemia and high-risk myelodysplastic syndrome Available from: https://clinicaltrials.gov/ct2/show/NCT02641002. NLM identifier: NCT02641002Accessed March 28, 2018
  • Forty SevenA first-in-human phase 1 dose escalation trial of Hu5F9-G4 in patients with advanced solid malignancies Available from: https://clinicaltrials.gov/ct2/show/NCT02216409. NLM identifier: NCT02216409Accessed March 28, 2018
  • Trillium TherapeuticsA phase 1A/1B dose escalation and expansion trial of TTI-621, a novel biologic targeting CD47, in subjects with relapsed or refractory hematologic malignancies and selected solid tumors Available from: https://clinicaltrials.gov/ct2/show/NCT02663518. NLM identifier: NCT02663518Accessed March 28, 2018
  • CelgeneA phase I, open-label, dose finding study of CC-90002, a monoclonal antibody directed against CD47, in subjects with advanced solid and hematologic cancers Available from: https://clinicaltrials.gov/ct2/show/NCT02367196. NLM identifier: NCT02367196Accessed March 28, 2018
  • Alexo TherapeuticsA phase 1, dose escalation study of ALX148 in patients with advanced solid tumors and lymphoma Available from: https://clinicaltrials.gov/ct2/show/NCT03013218. NLM identifier: NCT03013218Accessed March 28, 2018
  • XiaSJPresseyJGBarrFGMolecular pathogenesis of rhabdomyosarcomaCancer Biol Ther200219710412170781
  • HuhWWSkapekSXChildhood rhabdomyosarcoma: new insight on biology and treatmentCurr Oncol Rep20101240241020820958
  • OgnjanovicSLinaberyAMCharbonneauBRossJATrends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975–2005Cancer20091154218422619536876
  • HinikerSMDonaldsonSSRecent advances in understanding and managing rhabdomyosarcomaF1000Prime Rep201575926097732
  • NewtonWAGehanEAWebberBLClassification of rhabdomyosarcomas and related sarcomas: pathologic aspects and proposal for a new classification – an Intergroup Rhabdomyosarcoma StudyCancer199576107310858625211
  • TerrierPHenry-AmarMTricheTJIs neural-ectodermal differentiation of Ewing’s sarcoma of bone associated with an unfavorable prognosis?Eur J Cancer199531A3073147786593
  • HosoiHCurrent status of treatment for pediatric rhabdomyosarcoma in the USA and JapanPediatr Int201658818726646016
  • MalempatiSHawkinsDSRhabdomyosarcoma: review of the Children’s Oncology Group (COG) Soft-Tissue Sarcoma Committee experience and rationale for current COG studiesPediatr Blood Cancer20125951022378628
  • ShernJFChenLChmieleckiJComprehensive genomic analysis of rhabdomyosarcoma reveals a landscape of alterations affecting a common genetic axis in fusion-positive and fusion-negative tumorsCancer Discov2014421623124436047
  • HuhWWSkapekSXChildhood rhabdomyosarcoma: new insight on biology and treatmentCurr Oncol Rep20101240241020820958
  • SorensenPHLynchJCQualmanSJPAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the Children’s Oncology GroupJ Clin Oncol2002202672267912039929
  • FredericksWJGaliliNMukhopadhyaySThe PAX3-FKHR fusion protein created by the t(2;13) translocation in alveolar rhabdomyosarcomas is a more potent transcriptional activator than PAX3Mol Cell Biol199515152215357862145
  • AndersonMJSheltonGDCaveneeWKArdenKCEmbryonic expression of the tumor-associated PAX3-FKHR fusion protein interferes with the developmental functions of Pax3Proc Natl Acad Sci U S A2001981589159411171995
  • EpsteinJASongBLakkisMWangCTumor-specific PAX3-FKHR transcription factor, but not PAX3, activates the platelet-derived growth factor alpha receptorMol Cell Biol199818411841309632796
  • LagutinaIConwaySJSublettJGrosveldGFPax3-FKHR knock-in mice show developmental aberrations but do not develop tumorsMol Cell Biol2002227204721612242297
  • CroseLEGalindoKAKephartJGAlveolar rhabdomyosarcomaassociated PAX3-FOXO1 promotes tumorigenesis via Hippo pathway suppressionJ Clin Invest201412428529624334454
  • LinardicCMNainiSHerndonJE2ndKesserwanCQualmanSJCounterCMThe PAX3-FKHR fusion gene of rhabdomyosarcoma cooperates with loss of p16INK4A to promote bypass of cellular senescenceCancer Res2007676691669917638879
  • WilliamsonDMissiagliaEde ReyniesAFusion gene-negative alveolar rhabdomyosarcoma is clinically and molecularly indistinguishable from embryonal rhabdomyosarcomaJ Clin Oncol2010282151215820351326
  • BarrFGDuanFSmithLMGenomic and clinical analyses of 2p24 and 12q13–14 amplification in alveolar rhabdomyosarcoma: a report from the Children’s Oncology GroupGenes Chromosomes Cancer20094866167219422036
  • LohWEJrScrableHJLivanosEHuman chromosome 11 contains two different growth suppressor genes for embryonal rhabdomyosarcomaProc Natl Acad Sci U S A199289175517591347425
  • Weber-HallSAndersonJMcManusAGains, losses, and amplification of genomic material in rhabdomyosarcoma analyzed by comparative genomic hybridizationCancer Res199656322032248764111
  • BridgeJALiuJWeiboltVNovel genomic imbalances in embryonal rhabdomyosarcoma revealed by comparative genomic hybridization and fluorescence in situ hybridization: an intergroup rhabdomyosarcoma studyGenes Chromosomes Cancer20002733734410719362
  • GordonTMcManusAAndersonJCytogenetic abnormalities in 42 rhabdomyosarcoma: a United Kingdom Cancer Cytogenetics Group StudyMed Pediatr Oncol20013625926711452933
  • StrattonMRFisherCGustersonBACooperCSDetection of point mutations in N-ras and K-ras genes of human embryonal rhabdomyosarcomas using oligonucleotide probes and the polymerase chain reactionCancer Res198949632463272680062
  • PresseyJGAndersonJRCrossmanDKLynchJCBarrFGHedgehog pathway activity in pediatric embryonal rhabdomyosarcoma and undifferentiated sarcoma: a report from the Children’s Oncology GroupPediatr Blood Cancer20115793093821618411
  • PaulsonVChandlerGRakhejaDHigh-resolution array CGH identifies common mechanisms that drive embryonal rhabdomyosarcoma pathogenesisGenes Chromosomes Cancer20115039740821412928
  • ShuklaNAmeurNYilmazIOncogene mutation profiling of pediatric solid tumors reveals significant subsets of embryonal rhabdomyosarcoma and neuroblastoma with mutated genes in growth signaling pathwaysClin Cancer Res20121874875722142829
  • TakahashiYOdaYKawaguchiKAltered expression and molecular abnormalities of cell-cycle-regulatory proteins in rhabdomyosarcomaMod Pathol20041766066915098008
  • TaylorACShuLDanksMKP53 mutation and MDM2 amplification frequency in pediatric rhabdomyosarcoma tumors and cell linesMed Pediatr Oncol2000359610310918230
  • HawkinsDSGuptaAARudzinskiERWhat is new in the biology and treatment of pediatric rhabdomyosarcoma?Curr Opin Pediatr201426505624326270
  • HuangFGreerAHurlburtWThe mechanisms of differential sensitivity to an insulin-like growth factor-1 receptor inhibitor (BMS-536924) and rationale for combining with EGFR/HER2 inhibitorsCancer Res20096916117019117999
  • RenshawJTaylorKRBishopRDual blockade of the PI3K/AKT/mTOR (AZD8055) and RAS/MEK/ERK (AZD6244) pathways synergistically inhibits rhabdomyosarcoma cell growth in vitro and in vivoClin Cancer Res2013195940595123918606
  • GuentherMKGraabUFuldaSSynthetic lethal interaction between PI3K/Akt/mTOR and Ras/MEK/ERK pathway inhibition in rhabdomyosarcomaCancer Lett201333720020923684925
  • ChenXStewartEShelatAATargeting oxidative stress in embryonal rhabdomyosarcomaCancer Cell20132471072424332040
  • ZhangMLinardicCMKirschDGRAS and ROS in rhabdomyosarcomaCancer Cell20132468969124332036
  • MerchantMSBernsteinDAmoakoMAdjuvant immunotherapy to improve outcome in high risk pediatric sarcomasClin Cancer Res2016223182319126823601
  • National Cancer InstituteA pilot study of autologous T-cell transplantation with vaccine driven expansion of anti-tumor effectors after cytoreductive therapy in metastatic pediatric sarcomas Available from: https://clinicaltrials.gov/ct2/show/NCT00001566. NLM identifier: NCT00001566Accessed March 28, 2018
  • National Cancer InstituteA pilot study of tumor vaccination and R-hIL-7 following standard multimodality therapy in patients with high risk pediatric solid tumors Available from: https://clinicaltrials.gov/ct2/show/NCT00923351. NLM identifier: NCT00923351Accessed March 28, 2018
  • Jennerex BiotherapeuticsA phase I, open-label, dose escalation study of JX-594 (vaccinia GM-CSF/thymidine kinase-deactivated virus) administered by intratumoral injection in pediatric patients with unresectable refractory solid tumors Available from: https://clinical-trials.gov/ct2/show/NCT01169584. NLM identifier: NCT01169584Accessed March 28, 2018
  • Nationwide Children’s HospitalA phase I dose escalation study of intratumoral or intravenous herpes simplex virus-1 mutant HSV1716 in patients with refractory non-central nervous system (non-CNS) solid tumors Available from: https://clinicaltrials.gov/ct2/show/NCT00931931. NLM identifier: NCT00931931Accessed March 28, 2018
  • National Cancer InstituteA pilot study of tumor vaccination and R-hIL-7 following standard multimodality therapy in patients with high risk pediatric solid tumors Available from: https://clinicaltrials.gov/ct2/show/NCT00923351. NLM identifier: NCT00923351Accessed March 28, 2018
  • Baylor College of MedicineTumor associated antigen (TAA)-specific cytotoxic T-lymphocytes administered to patients with solid tumors Available from: https://clinicaltrials.gov/ct2/show/NCT02239861. NLM identifier: NCT02239861Accessed March 28, 2018
  • Children’s Oncology GroupA phase 2 study of IMGN901 (Lor-votuzumab Mertansine; NSC: 783609) in children with relapsed or refractory Wilms tumor, rhabdomyosarcoma, neuroblastoma, pleuropulmonary blastoma, malignant peripheral nerve sheath tumor (MPNST) and synovial sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT02452554. NLM identifier: NCT02452554Accessed March 28, 2018
  • De AlavaEKawaiAHealeyJHEWS-FLI1 fusion transcript structure is an independent determinant of prognosis in Ewing’s sarcomaJ Clin Oncol199816124812559552022
  • CotterillSJAhrensSPrognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma Study groupJ Clin Oncol2000181703108311410963639
  • AngervallLEnzingerFMExtraskeletal neoplasm resembling Ewing’s sarcomaCancer19753612402511203852
  • SorensenPHLessnickSLLopez-TerradaDLiuXFTricheTJDennyCTA second Ewing’s sarcoma translocation, t(21;22), fuses the EWS gene to another ETS-family transcription factor, ERGNat Genet199461461518162068
  • SiebenrockKANascimentoAGRockMGComparison of soft tissue Ewing’s sarcoma and peripheral neuroectodermal tumorClin Orthop Relat Res1996288299
  • TanakaMYamazakiYEwing’s sarcoma precursors are highly enriched in embryonic osteochondrogenic progenitorsJ Clin Invest20141243061307424911143
  • RiggiNStamenkovicIThe biology of Ewing sarcomaCancer Lett200725411017250957
  • van DoominckJAJiLSchaubBCurrent treatment protocols have eliminated the prognostic advantage of type 1 fusions in Ewing sarcoma: a report from the Children’s Oncology GroupJ Clin Oncol2010281989199420308669
  • CromptonBDStewartCTaylor-WeinerAThe genomic landscape of pediatric Ewing sarcomaCancer Discov201441326134125186949
  • TirodeFSurdezDMaXGenomic landscape of Ewing sarcoma defines an aggressive subtype with co-association of STAG2 and TP53 mutationsCancer Discov201441342135325223734
  • MayWALessnickSLBraunBSThe Ewing’s sarcoma EWS/FLI-1 fusion gene encodes a more potent transcriptional activator and is a more powerful transforming gene than FLI-1Mol Cell Biol199313739373988246959
  • JohnsonKMMahlerNRSaundRSRole for the EWS domain of EWS/FLI in binding GGAA-microsatellites required for Ewing sarcoma anchorage independent growthProc Natl Acad Sci U S A20171149870987528847958
  • KinseyMSmithRLessnickSLNR0B1 is required for the oncogenic phenotype mediated by EWS/FLI in Ewing’s sarcomaMol Cancer Res2006485185917114343
  • LuQZhangYMaLEWS-FLI1 positively regulates autophagy by increasing ATG4B expression in Ewing sarcoma cellsInt J Mol Med2017401217122528902354
  • HeTSurdexDRantalaJKHigh-throughput RNAi screen in Ewing sarcoma cells identifies leucine rich repeats and WD repeat domain containing 1 (LRWD1) as a regulator of EWS-FLI1 driven cell viabilityGene201759613714627760381
  • TannerJMBensardCWeiPEWS/FLI is a master regulator of metabolic reprogramming in Ewing sarcomaMol Cancer Res2017151517153028720588
  • ChooSWangPNewburyRRobertsWYangJReactivation of TWIST1 contributes to Ewing sarcoma metastasisPediatric Blood Cancer201865e26721
  • SechlerMParrishJKBirksDKJedlickaPThe histone demethylase KDM3A, and its downstream target MCAM, promote Ewing sarcoma cell migration and metastasisOncogene2017364150416028319067
  • PetersHLYanYNordgrenTMCutucacheCEJoshiSSSolheimJCAmyloid precursor like protein 2 suppresses irradiation induced apoptosis in Ewing sarcoma cells and is elevated in immune evasive Ewing sarcoma cellsCancer Biol Ther20131475276023792571
  • SubbiahVAndersonPLazarAJBurdettERaymondKLudwigJAEwing’s sarcoma: standard and experimental treatment optionsCurr Treat Options Oncol20091012614019533369
  • KovarHBlocking the road, stopping the engine or killing the driver? Advances in targeting EWS/FLI-1 fusion in Ewing sarcoma as novel therapyExpert Opin Ther Targets2014181315132825162919
  • AksnesLHBauerHCDahlAAHealth status at long-term follow-up in patients treated for extremity localized Ewing sarcoma or osteosarcoma: a Scandinavian Sarcoma Group studyPediatr Blood Cancer200953848919343771
  • ParksRRaschEKManskyPJOakleyFDifferences in activities of daily living performance between long-term pediatric sarcoma survivors and a matched comparison group on standardized testingPediatr Blood Cancer20095362262819533662
  • Oncternal TherapeuticsTK216 in patients with relapsed or refractory Ewing sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT02657005. NLM identifier: NCT02657005Accessed March 28, 2018
  • ZöllnerSKSelvanathanSPGrahamGTInhibition of the oncogenic fusion protein EWS-FLI1 causes G2-M cell cycle arrest and enhanced vincristine sensitivity in Ewing’s sarcomaSci Signal201710eaam842928974650
  • SpurnyCKailayangiriSJamitzkySProgrammed cell death ligand 1 (PD-L1) expression is not a predominant feature in Ewing sarcomasPediatr Blood Cancer201865e26719
  • GroharPJGriffinLBYeungCEcteinascidin 743 interferes with the activity of EWS-FLI1 in Ewing sarcoma cellsNeoplasia20111314515321403840
  • BaruchelSPappoAKrailoMA phase 2 trial of trabectedin in children with recurrent rhabdomyosarcoma, Ewing sarcoma and non-rhabdomyosarcoma soft tissue sarcomas: a report from the Children’s Oncology GroupEur J Cancer20124857958522088484
  • GroharPJSegarsLEYeungCDual targeting of EWS-FLI1 activity and the associated DNA damage response with trabectedin and SN38 synergistically inhibits Ewing sarcoma cell growthClin Cancer Res2014201190120324277455
  • ErkizanHVKongYMerchantMA small molecule blocking oncogenic protein EWS-FLI1 interaction with RNA helicase A inhibits growth of Ewing’s sarcomaNat Med20091575075619584866
  • PrieurATirodeFCohenPDelattreOEWS/FLI-1 silencing and gene profiling of Ewing cells reveal downstream oncogenic pathways and a crucial role for repression of insulin-like growth factor binding protein 3Mol Cell Biol2004247275728315282325
  • MalempatiSWeigelBIngleAMPhase I/II trial and pharmacokinetic study of cixutumumab in pediatric patients with refractory solid tumors and Ewing sarcoma: a report from the Children’s Oncology GroupJ Clin Oncol20123025626222184397
  • PappoASPatelSRCrowleyJR1507, a monoclonal antibody to the insulin-like growth factor 1 receptor, in patients with recurrent or refractory Ewing sarcoma family of tumors: results of a phase II Sarcoma Alliance for Research Through Collaboration studyJ Clin Oncol2011294541454722025149
  • Lamhamedi-CherradiSEMenegazBARamamoorthyVIGF-1R and mTOR blockade: novel resistance mechanisms and synergistic drug combinations for Ewing sarcomaJ Natl Cancer Inst2016108djw18227576731
  • NaingALoRussoPFuSInsulin growth factor-receptor (IGF-1R) antibody cixutumumab combined with the mTOR inhibitor temsirolimus in patients with refractory Ewing’s sarcoma family tumorsClin Cancer Res2012182625263122465830
  • GarnettMJEdelmanEJHeidornSJSystematic identification of genomic markers of drug sensitivity in cancer cellsNature201248357057522460902
  • HeskeCMDavisMIBaumgartJTMatrix Screen identifies synergistic combination of PARP inhibitors and nicotinamide phosphoribosyltransferase (NAMPT) inhibitors in Ewing sarcomaClin Cancer Res2017237301731128899971
  • ChoyEButrynskiJEHarmonDCPhase II study of olaparib in patients with refractory Ewing sarcoma following failure of standard chemotherapyBMC Cancer20141481325374341
  • National Cancer InstituteNCI-COG pediatric MATCH (molecular analysis for therapy choice): a phase 2 subprotocol of olaparib in patients with tumors harboring defects in DNA damage repair genes Available from: https://clinicaltrials.gov/ct2/show/NCT03233204. NLM identifier: NCT03233204Accessed March 28, 2018
  • Massachusetts General HospitalPhase I study of olaparib and temozolomide in adult patients with recurrent/metastatic Ewing’s sarcoma following failure of prior chemotherapy Available from: https://clinicaltrials.gov/ct2/show/NCT01858168. NLM identifier: NCT01858168Accessed March 28, 2018
  • RajSBuiMGonzalesRLetsonDAntoniaSJImpact of PDL1 expression on clinical outcomes in subtypes of sarcomaAnn Oncol201425iv494iv510
  • LeDTUramJNWangHPD-1 blockade in tumors with mismatch-repair deficiencyN Engl J Med20153722509252026028255
  • Assaf-Harofeh Medical CenterA phase II single arm study assessing efficacy and safety of nivolumab plus ipilimumab in nonresectable/metastatic sarcoma and endometrial carcinoma patients with somatic deficient MMR as a selection tool Available from: https://clinicaltrials.gov/ct2/show/NCT02982486. NLM identifier: NCT02982486Accessed March 28, 2018
  • von HeykingKCalzada-WackJGollnerSThe endochondral bone protein CHM1 sustains an undifferentiated, invasive phenotype, promoting lung metastasis in Ewing sarcomaMol Oncol2017111288130128319320
  • ThielUPirsonSMüller-SpahnCSpecific recognition and inhibition of Ewing tumor growth by antigen-specific allo-restricted cytotoxic T cellsBr J Cancer201110494895621407224
  • BlaeschkeFThielUKirschnerAHuman HLA-A 02-01/CHM1+ allo-restricted T cell receptor transgenic CD8+ T cells specifically inhibit Ewing sarcoma growth in vitro and in vivoOncotarget20167432674328027281613
  • GhisoliMBarveMSchneiderRPilot Trial of FANG immu-notherapy in Ewing’s sarcomaMol Ther2015231103110925917459
  • GradalisA two-part phase IIB trial of Vigil (Bi-shRNA-furin and GMCSF augmented autologous tumor cell immunotherapy) in Ewing’s sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT02511132. NLM identifier: NCT02511132Accessed March 28, 2018
  • National Cancer InstituteA pilot study of tumor vaccination and R-hIL-7 following standard multimodality therapy in patients with high risk pediatric solid tumors Available from: https://clinicaltrials.gov/ct2/show/NCT00923351. NLM identifier: NCT00923351Accessed March 28, 2018
  • SpurrellELFisherCThomasJMJudsonIRPrognostic factors in advanced synovial sarcoma: an analysis of 104 patients treated at the Royal Marsden HospitalAnn Oncol20051643744415653701
  • NielsenTOPoulinNMLadanyiMSynovial sarcoma: recent discoveries as a roadmap to new avenues for therapyCancer Discov2015512413425614489
  • HerzogCEOverview of sarcomas in the adolescent and young adult populationJ Pediatr Hematol Oncol200527215815838394
  • LadanyiMAntonescuCRLeungDHImpact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patientsCancer Res20026213514011782370
  • SultanIRodriguez-GalindoCSaabRYasirSCasanovaMFerrariAComparing children and adults with synovial sarcoma in the Surveillance, Epidemiology, and End Results program, 1983 to 2005:an analysis of 1268 patientsCancer20091153537354719514087
  • FisherCSynovial sarcomaAnn Diagn Pathol199824014219930576
  • ClarkJRocquesPJCrewAJIdentification of novel genes, SYT and SSX, involved in the t(X;18)(p11.2;q11.2) translocation found in human synovial sarcomaNat Genet199475025087951320
  • NagayamaSKatagiriTTsunodaTGenome-wide analysis of gene expression in synovial sarcomas using a cDNA microarrayCancer Res2002625859586612384549
  • WeissSWSobinLHHistological Typing of Soft Tissue Tumours2nd edHeidelbergSpringer1994
  • ClarkJRocquesPJCrewAJIdentification of novel genes, SYT and SSX, involved in the t(X;18)(p11.2;q11.2) translocation found in human synovial sarcomaNat Genet199475025087951320
  • SkyttingBNilssonGBrodinBA novel fusion gene, SYT-SSX4, in synovial sarcomaJ Natl Cancer Inst19999197497510359553
  • KawaiAWoodruffJHealeyJHBrennanMFAntonescuCRLadanyiMSYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcomaN Engl J Med19983381531609428816
  • AntonescuCRKawaiALeungDHStrong association of SYT-SSX fusion type and morphologic epithelial differentiation in synovial sarcomaDiagn Mol Pathol200091810718206
  • NakaqawaYNumotoKYoshidaAChromosomal and genetic imbalances in synovial sarcoma detected by conventional and microarray comparative genomic hybridizationJ Cancer Res Clin Oncol200613244445016557383
  • SzymanskaJSerraMSkyttingBGenetic imbalances in 67 synovial sarcomas evaluated by comparative genomic hybridizationGenes Chromosomes Cancer1998232132199790501
  • AllanderSVIlleiPBChenYExpression profiling synovial sarcoma by cDNA microarrays: association of ERBB2, IGFBP2, and ELF3 with epithelial differentiationAm J Pathol20021611587159512414507
  • SaitoTOdaYSakamotoAPrognostic value of the preserved expression of the E-cadherin and catenin families of adhesion molecules and of B-catenin mutations in synovial sarcomaJ Pathol200019234235011054718
  • NielsenTHsuFDO’ConnellJTissue microarray validation of epidermal growth factor receptor and SALL2 in synovial sarcoma with comparison to tumors of similar histologyAm J Pathol20031631449145614507652
  • TerryJSaitoTSubramanianSTLE1 as a diagnostic immunohistochemical marker for synovial sarcoma emerging from gene expression profiling studiesAm J Surg Pathol20073124024617255769
  • Washington University School of MedicineA phase I/II clinical trial of dose-escalating DHEA in synovial sarcoma patients Available from: https://clinicaltrials.gov/ct2/show/NCT02683148. NLM identifier: NCT02683148Accessed March 28, 2018
  • EpizymeA phase 1 study of the EZH2 inhibitor tazemetostat in pediatric subjects with relapsed or refractory INI1-negative tumors or synovial sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT02601937. NLM identifier: NCT02601937Accessed March 28, 2018
  • EpizymeA phase II, multicenter study of the EZH2 inhibitor tazemetostat in adult subjects with INI1-negative tumors or relapsed/refractory synovial sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT02601950. NLM identifier: NCT02601950Accessed March 28, 2018
  • Advenchen LaboratoriesA phase III study of AL3818 (anlotinib) hydrochloride monotherapy in subjects with metastatic or advanced alveolar soft part sarcoma, leiomyosarcoma, and synovial sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT03016819. NLM identifier: NCT03016819Accessed March 28, 2018
  • National Cancer InstituteA phase 1B/2 study of imatinib in combination with everolimus in synovial sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT01281865. NLM identifier: NCT01281865Accessed March 28, 2018
  • Memorial Sloan Kettering Cancer CenterPhase II trial of sorafenib and dacarbazine in soft tissue sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT00837148. NLM identifier: NCT00837148Accessed March 28, 2018
  • KawaguchiSWadaTIdaKPhase I vaccination trial of SYT-SSX junction peptide in patients with disseminated synovial sarcomaJ Transl Med20053115647119
  • SuminoeAMatsuzakiAHattoriHKogaYHaraTImmunotherapy with autologous dendritic cells and tumor antigens for children with refractory malignant solid tumorsPediatr Transplant20091374675319067917
  • KawaguchiSTsukaharaTIdaKSYT-SSX breakpoint peptide vaccines in patients with synovial sarcoma: a study from the Japanese Musculoskeletal Oncology GroupCancer Sci20121031625163022726592
  • JungbluthAAAntonescuCRBusamKJMonophasic and biphasic synovial sarcomas abundantly express cancer/testis antigen NY-ESO-1 but not MAGE-A1 or CT7Int J Cancer20019425225611668506
  • RobbinsPFMorganRAFeldmanSATumor regression in patients with metastatic synovial cell sarcoma and melanoma using genetically engineered lymphocytes reactive with NY-ESO-1J Clin Oncol201129911924
  • JagerENagataYGnjaticSMonitoring CD8 T cell responses to NY-ESO-1: correlation of humoral and cellular immune responsesProc Natl Acad Sci U S A2000974760476510781081
  • StockertEJagerEChenYTA survey of the humoral immune response of cancer patients to a panel of human tumor antigensJ Exp Med1998187134913549547346
  • van den EyndeBJvan der BruggenPT cell defined tumor antigensCurr Opin Immunol199796846939368778
  • AdaptimmuneA pilot study of genetically engineered NY-ESO-1 specific NY-ESO-1 C259T in HLA-A2+ patients with synovial sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT01343043. NLM identifier: NCT01343043Accessed March 28, 2018
  • National Cancer InstitutePhase II study of metastatic cancer that expresses NY-ESO-1 using lymphodepleting conditioning followed by infusion of anti-NY-ESO-1 murine TCR-gene engineered lymphocytes Available from: https://clinicaltrials.gov/ct2/show/NCT01967823. NLM identifier: NCT01967823Accessed March 28, 2018
  • Takara BioMulti-center phase I/II study of NY-ESO-1 T cell receptor gene transferred T lymphocytes in patients with synovial sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT03250325. NLM identifier: NCT03250325Accessed March 28, 2018
  • Immune DesignA randomized, open-label, phase II trial of CMB305 (sequentially administered LV305 and G305) and atezolizumab in patients with locally advanced, relapsed, or metastatic sarcoma expressing NY-ESO-1 Available from: https://clinicaltrials.gov/ct2/show/NCT02609984. NLM identifier: NCT02609984Accessed March 28, 2018
  • Petrov Research Institute of OncologyNon-randomized single-center study phase II evaluating the efficacy and toxicity of autologous dendritic cell vaccine loaded with allogenic tumor lysate expression of cancer testis antigens in patients with soft tissue sarcoma Available from: https://clinicaltrials.gov/ct2/show/NCT01883518. NLM identifier: NCT01883518Accessed March 28, 2018
  • ZhangPPollockREEpigenetic regulators: new therapeutic targets for soft tissue sarcomaCancer Cell Microenviron20141e19126078988
  • KawaguchiKOdaYSaitoTDNA hypermethylation status of multiple genes in soft tissue sarcomasMod Pathol200614106114
  • LubienieckaJMde BruijnDRSuLHistone deacetylase inhibitors reverse SS18-SSX-mediated polycomb silencing of the tumor suppressor early growth response 1 in synovial sarcomaCancer Res2008684303431018519690
  • WangHGarzonRSunHNF-κB-YY1-miR-29 regulatory circuitry in skeletal myogenesis and rhabdomyosarcomaCancer Cell20081436938118977326