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dc.contributor.authorChew, Wen_US
dc.contributor.authorBenson, Cen_US
dc.contributor.authorThway, Ken_US
dc.contributor.authorHayes, Aen_US
dc.contributor.authorMiah, Aen_US
dc.contributor.authorZaidi, Sen_US
dc.contributor.authorLee, ATJen_US
dc.contributor.authorMessiou, Cen_US
dc.contributor.authorFisher, Cen_US
dc.contributor.authorvan der Graaf, WTen_US
dc.contributor.authorJones, RLen_US
dc.identifier.citationMedical oncology (Northwood, London, England), 2018, 35 (11), pp. 138 - ?en_US
dc.description.abstract<h4>Background</h4>Sclerosing epithelioid fibrosarcoma (SEF) is a very rare soft tissue sarcoma subtype. Clinically it is an aggressive tumour; however, to our knowledge there are no published reports regarding the efficacy of chemotherapy in SEF. Therefore, the aim of this study was to document the outcome of a series of patients with SEF treated at a single referral centre with reference to systemic therapy.<h4>Methods</h4>A retrospective search of a prospectively maintained database was performed to identify all patients diagnosed with SEF between 1990 and 2017. The diagnosis was confirmed in each case by a dedicated soft tissue sarcoma pathologist. We analysed those with recurrent disease and the effect of systemic chemotherapy in the metastatic setting.<h4>Results</h4>Thirteen patients were identified, median overall survival from diagnosis and metastasis were 47.3 (95% CI 25.0-131.9) and 16.3 (95% CI 5.3-20.6) months, respectively. In total, 12 (92.3%) patients developed metastatic disease of which 10 died of disease, 1 was lost to follow-up and 1 had recently commenced palliative treatment. Among the 10 patients with metastatic disease, 7 received palliative chemotherapy. Palliative chemotherapy resulted in partial response in 1 patient, stable disease in 3 patients and progressive disease in 3 patients. Median time to disease progression was 2.7 (95% CI 1.2-4.4) months. Two of 13 patients were treated with adjuvant chemotherapy, receiving 6 cycles of liposomal doxorubicin and 1 cycle of doxorubicin, respectively, with a metastasis-free survival of 28.2 and 7.1 months, respectively.<h4>Conclusion</h4>SEF is an aggressive sarcoma subtype with a poor outcome and with limited responsiveness to conventional chemotherapy. Patients with this subtype should be considered for participation in clinical trials with novel agents. Further investigation into the biology of this rare disease is required to improve outcomes.en_US
dc.format.extent138 - ?en_US
dc.subjectAntineoplastic Agentsen_US
dc.subjectAntineoplastic Combined Chemotherapy Protocolsen_US
dc.subjectTreatment Outcomeen_US
dc.subjectSurvival Rateen_US
dc.subjectRetrospective Studiesen_US
dc.subjectProspective Studiesen_US
dc.subjectMiddle Ageden_US
dc.titleClinical Characteristics and efficacy of chemotherapy in sclerosing epithelioid fibrosarcoma.en_US
dc.typeJournal Article
rioxxterms.typeJournal Article/Reviewen_US
dc.relation.isPartOfMedical oncology (Northwood, London, England)en_US
pubs.notesNot knownen_US
pubs.organisational-group/ICR/Primary Group
pubs.organisational-group/ICR/Primary Group/ICR Divisions
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Cancer Biology
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Cancer Biology/Targeted Therapy
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies/Clinical and Translational Sarcoma
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/ICR Divisions/Radiotherapy and Imaging
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Radiotherapy and Imaging/Sarcoma and Melanoma Surgery
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Radiotherapy and Imaging/Targeted Therapy
pubs.organisational-group/ICR/Primary Group/Royal Marsden Clinical Units
pubs.embargo.termsNot knownen_US
icr.researchteamClinical and Translational Sarcomaen_US
icr.researchteamSarcoma Clinical Trials (R Jones)en_US
icr.researchteamSarcoma and Melanoma Surgeryen_US
icr.researchteamTargeted Therapyen_US
dc.contributor.icrauthorFisher, Cyrilen
dc.contributor.icrauthorHayes, Andrewen
dc.contributor.icrauthorZaidi, Shane Haideren
dc.contributor.icrauthorMessiou, Christinaen
dc.contributor.icrauthorJones, Robinen
dc.contributor.icrauthorvan der Graaf, Wilhelminaen

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