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Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study

dc.contributor.authorFrezza, AM
dc.contributor.authorCesari, M
dc.contributor.authorBaumhoer, D
dc.contributor.authorBiau, D
dc.contributor.authorBielack, S
dc.contributor.authorCampanacci, DA
dc.contributor.authorCasanova, J
dc.contributor.authorEsler, C
dc.contributor.authorFerrari, S
dc.contributor.authorFunovics, PT
dc.contributor.authorGerrand, C
dc.contributor.authorGrimer, R
dc.contributor.authorGronchi, A
dc.contributor.authorHaffner, N
dc.contributor.authorHecker-Nolting, S
dc.contributor.authorHöller, S
dc.contributor.authorJeys, L
dc.contributor.authorJutte, P
dc.contributor.authorLeithner, A
dc.contributor.authorSan-Julian, M
dc.contributor.authorThorkildsen, J
dc.contributor.authorVincenzi, B
dc.contributor.authorWindhager, R
dc.contributor.authorWhelan, J
dc.date.accessioned2016-05-12T10:10:36Z
dc.date.available2016-05-12T10:10:36Z
dc.date.issued2015-02
dc.description.abstractBACKGROUND: Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcoma with little evidence supporting treatment recommendations. PATIENTS AND METHODS: Specialist centres collaborated to report prognostic factors and outcome for 113 patients. RESULTS: Median age was 30 years (range: 11-80), male/female ratio 1.1. Primary sites were extremities (40%), trunk (47%) and head and neck (13%), 41 arising primarily in soft tissue. Seventeen patients had metastases at diagnosis. Mean follow-up was 14.9 years (range: 1-34), median overall survival (OS) 17 years (95% confidence interval (CI): 10.3-28.6). Ninety-five of 96 patients with localised disease underwent surgery, 54 additionally received combination chemotherapy. Sixty-five of 95 patients are alive and 45 progression-free (5 local recurrence, 34 distant metastases, 11 combined). Median progression-free survival (PFS) and OS were 7 (95% CI: 3.03-10.96) and 20 (95% CI: 12.63-27.36) years respectively. Chemotherapy administration in patients with localised disease was associated with reduced risk of recurrence (P=0.046; hazard ratio (HR)=0.482 95% CI: 0.213-0.996) and death (P=0.004; HR=0.445 95% CI: 0.256-0.774). Clear resection margins predicted less frequent local recurrence (2% versus 27%; P=0.002). Primary site and origin did not influence survival. The absence of metastases at diagnosis was associated with a significantly better outcome (P<0.0001). Data on radiotherapy indications, dose and fractionation were insufficiently complete, to allow comment of its impact on outcomes. Median OS for patients with metastases at presentation was 3 years (95% CI: 0-4.25). CONCLUSIONS: Prognosis in MCS varies considerably. Metastatic disease at diagnosis has the strongest impact on survival. Complete resection and adjuvant chemotherapy should be considered as standard of care for localised disease.pt_PT
dc.identifier.citationEur J Cancer. 2015 Feb;51(3):374-81.pt_PT
dc.identifier.doi10.1016/j.ejca.2014.11.007pt_PT
dc.identifier.urihttp://hdl.handle.net/10400.4/1921
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.subjectNeoplasias dos Ossospt_PT
dc.subjectCondrossarcoma Mesenquimalpt_PT
dc.titleMesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society studypt_PT
dc.typejournal article
dspace.entity.typePublication
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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