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dc.contributor.authorFrezza, AMen_US
dc.contributor.authorJones, RLen_US
dc.contributor.authorLo Vullo, Sen_US
dc.contributor.authorAsano, Nen_US
dc.contributor.authorLucibello, Fen_US
dc.contributor.authorBen-Ami, Een_US
dc.contributor.authorRatan, Ren_US
dc.contributor.authorTeterycz, Pen_US
dc.contributor.authorBoye, Ken_US
dc.contributor.authorBrahmi, Men_US
dc.contributor.authorPalmerini, Een_US
dc.contributor.authorFedenko, Aen_US
dc.contributor.authorVincenzi, Ben_US
dc.contributor.authorBrunello, Aen_US
dc.contributor.authorDesar, IMEen_US
dc.contributor.authorBenjamin, RSen_US
dc.contributor.authorBlay, JYen_US
dc.contributor.authorBroto, JMen_US
dc.contributor.authorCasali, PGen_US
dc.contributor.authorGelderblom, Hen_US
dc.contributor.authorGrignani, Gen_US
dc.contributor.authorGronchi, Aen_US
dc.contributor.authorHall, KSen_US
dc.contributor.authorMir, Oen_US
dc.contributor.authorRutkowski, Pen_US
dc.contributor.authorWagner, AJen_US
dc.contributor.authorAnurova, Oen_US
dc.contributor.authorCollini, Pen_US
dc.contributor.authorDei Tos, APen_US
dc.contributor.authorFlucke, Uen_US
dc.contributor.authorHornick, JLen_US
dc.contributor.authorLobmaier, Ien_US
dc.contributor.authorPhilippe, Ten_US
dc.contributor.authorPicci, Pen_US
dc.contributor.authorRanchere, Den_US
dc.contributor.authorRenne, SLen_US
dc.contributor.authorSbaraglia, Men_US
dc.contributor.authorThway, Ken_US
dc.contributor.authorWagrodzki, Men_US
dc.contributor.authorWang, W-Len_US
dc.contributor.authorYoshida, Aen_US
dc.contributor.authorMariani, Len_US
dc.contributor.authorKawai, Aen_US
dc.contributor.authorStacchiotti, Sen_US
dc.coverage.spatialUnited Statesen_US
dc.date.accessioned2018-11-28T10:01:42Z
dc.date.issued2018-09-01en_US
dc.identifierhttps://www.ncbi.nlm.nih.gov/pubmed/29800950en_US
dc.identifier2678088en_US
dc.identifier.citationJAMA Oncol, 2018, 4 (9), pp. e180219 - ?en_US
dc.identifier.urihttps://repository.icr.ac.uk/handle/internal/2959
dc.identifier.eissn2374-2445en_US
dc.identifier.doi10.1001/jamaoncol.2018.0219en_US
dc.description.abstractImportance: Epithelioid sarcoma (ES) is an exceedingly rare malignant neoplasm with distinctive pathologic, molecular, and clinical features as well as the potential to respond to new targeted drugs. Little is known on the activity of anthracycline-based regimens, gemcitabine-based regimens, and pazopanib in this disease. Objective: To report on the activity of anthracycline-based regimens, gemcitabine-based regimens, and pazopanib in patients with advanced ES. Design, Setting, and Participants: Seventeen sarcoma reference centers in Europe, the United States, and Japan contributed data to this retrospective analysis of patients with locally advanced/metastatic ES diagnosed between 1990 and 2016. Local pathological review was performed in all cases to confirm diagnosis according to most recent criteria. Exposures: All patients included in the study received anthracycline-based regimens, gemcitabine-based regimens, or pazopanib. Main Outcome and Measures: Response was assessed by RECIST. Progression-free survival (PFS) and overall survival (OS) were computed by Kaplan-Meier method. Classic and proximal subtypes were defined based on morphology (according to 2013 World Health Organization guidelines). Results: Overall, 115 patients were included, 80 (70%) were men and 35 (30%) were women, with a median age of 32 years (range, 15-77 years). Of the 115 patients with ES, 85 were treated with anthracycline-based regimens, 41 with gemcitabine-based regimens, and 18 with pazopanib. Twenty-four received more than 1 treatment. Median follow-up was 34 months. Response rate for anthracycline-based regimens was 22%, with a median PFS of 6 months. One complete response (CR) was reported. A trend toward a higher response rate was noticed in morphological proximal type (26%) vs classic type (19%) and in proximal vs distal primary site (26% vs 18%). The response rate for gemcitabine-based regimens was 27%, with 2 CR and a median PFS of 4 months. In this group, a trend toward a higher response rate was reported in classic vs proximal morphological type (30% vs 22%) and in distal vs proximal primary site (40% vs 14%). In the pazopanib group, no objective responses were seen, and median PFS was 3 months. Conclusions and Relevance: This is the largest retrospective series of systemic therapy in ES. We confirm a moderate activity of anthracycline-based and gemcitabine-based regimens in ES, with a similar response rate and PFS in both groups. The value of pazopanib was low. These data may serve as a benchmark for trials of novel agents in ES.en_US
dc.format.extente180219 - ?en_US
dc.languageengen_US
dc.language.isoengen_US
dc.rights.urihttp://www.rioxx.net/licenses/under-embargo-all-rights-reserveden_US
dc.titleAnthracycline, Gemcitabine, and Pazopanib in Epithelioid Sarcoma: A Multi-institutional Case Series.en_US
dc.typeJournal Article
rioxxterms.versionofrecord10.1001/jamaoncol.2018.0219en_US
rioxxterms.licenseref.startdate2018-09-01en_US
rioxxterms.typeJournal Article/Reviewen_US
dc.relation.isPartOfJAMA Oncolen_US
pubs.issue9en_US
pubs.notesNot knownen_US
pubs.organisational-group/ICR
pubs.organisational-group/ICR/Primary Group
pubs.organisational-group/ICR/Primary Group/ICR Divisions
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies/Sarcoma Clinical Trials (R Jones)
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies/Sarcoma Clinical Trials (R Jones)/Sarcoma Clinical Trials (R Jones) (hon.)
pubs.organisational-group/ICR/Primary Group/Royal Marsden Clinical Units
pubs.publication-statusPublisheden_US
pubs.volume4en_US
pubs.embargo.termsNot knownen_US
icr.researchteamSarcoma Clinical Trials (R Jones)en_US
dc.contributor.icrauthorJones, Robinen_US


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