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dc.contributor.authorFrezza, AM
dc.contributor.authorRavi, V
dc.contributor.authorLo Vullo, S
dc.contributor.authorVincenzi, B
dc.contributor.authorTolomeo, F
dc.contributor.authorChen, TW-W
dc.contributor.authorTeterycz, P
dc.contributor.authorBaldi, GG
dc.contributor.authorItaliano, A
dc.contributor.authorPenel, N
dc.contributor.authorBrunello, A
dc.contributor.authorDuffaud, F
dc.contributor.authorHindi, N
dc.contributor.authorIwata, S
dc.contributor.authorSmrke, A
dc.contributor.authorFedenko, A
dc.contributor.authorGelderblom, H
dc.contributor.authorVan Der Graaf, W
dc.contributor.authorVozy, A
dc.contributor.authorConnolly, E
dc.contributor.authorGrassi, M
dc.contributor.authorBenjamin, RS
dc.contributor.authorBroto, J-M
dc.contributor.authorGrignani, G
dc.contributor.authorJones, RL
dc.contributor.authorKawai, A
dc.contributor.authorTysarowski, A
dc.contributor.authorMariani, L
dc.contributor.authorCasali, PG
dc.contributor.authorStacchiotti, S
dc.date.accessioned2021-06-11T13:29:38Z
dc.date.available2021-06-11T13:29:38Z
dc.identifier.citationCancer medicine, 2021, 10 (8), pp. 2645 - 2659
dc.identifier.issn2045-7634
dc.identifier.urihttps://repository.icr.ac.uk/handle/internal/4641
dc.identifier.eissn2045-7634
dc.identifier.doi10.1002/cam4.3807
dc.description.abstractBackground This observational, retrospective effort across Europe, US, Australia, and Asia aimed to assess the activity of systemic therapies in EHE, an ultra-rare sarcoma, marked by WWTR1-CAMTA1 or YAP1-TFE3 fusions.Methods Twenty sarcoma reference centres contributed data. Patients with advanced EHE diagnosed from 2000 onwards and treated with systemic therapies, were selected. Local pathologic review and molecular confirmation were required. Radiological response was retrospectively assessed by local investigators according to RECIST. Progression free survival (PFS) and overall survival (OS) were estimated by Kaplan-Meier method.Results Overall, 73 patients were included; 21 had more than one treatment. Thirty-three patients received anthracyclines regimens, achieving 1 (3%) partial response (PR), 25 (76%) stable disease (SD), 7 (21%) progressive disease (PD). The median (m-) PFS and m-OS were 5.5 and 14.3 months respectively. Eleven patients received paclitaxel, achieving 1 (9%) PR, 6 (55%) SD, 4 (36%) PD. The m-PFS and m-OS were 2.9 and 18.6 months, respectively. Twelve patients received pazopanib, achieving 3 (25%) SD, 9 (75%) PD. The m-PFS and m-OS were.2.9 and 8.5 months, respectively. Fifteen patients received INF-α 2b, achieving 1 (7%) PR, 11 (73%) SD, 3 (20%) PD. The m-PFS and m-OS were 8.9 months and 64.3, respectively. Among 27 patients treated with other regimens, 1 PR (ifosfamide) and 9 SD (5 gemcitabine +docetaxel, 2 oral cyclophosphamide, 2 others) were reported.Conclusion Systemic therapies available for advanced sarcomas have limited activity in EHE. The identification of new active compounds, especially for rapidly progressive cases, is acutely needed.
dc.formatPrint-Electronic
dc.format.extent2645 - 2659
dc.languageeng
dc.language.isoeng
dc.rights.urihttps://creativecommons.org/licenses/by/4.0
dc.titleSystemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature.
dc.typeJournal Article
dcterms.dateAccepted2021-02-09
rioxxterms.versionVoR
rioxxterms.versionofrecord10.1002/cam4.3807
rioxxterms.licenseref.urihttps://creativecommons.org/licenses/by/4.0
rioxxterms.typeJournal Article/Review
dc.relation.isPartOfCancer medicine
pubs.issue8
pubs.notesNot known
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.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-statusPublished
pubs.volume10
pubs.embargo.termsNot known
icr.researchteamSarcoma Clinical Trials (R Jones)
icr.researchteamSarcoma Clinical Trials (R Jones)en_US
dc.contributor.icrauthorJones, Robinen


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