Publication
Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study
dc.contributor.author | Frezza, AM | |
dc.contributor.author | Cesari, M | |
dc.contributor.author | Baumhoer, D | |
dc.contributor.author | Biau, D | |
dc.contributor.author | Bielack, S | |
dc.contributor.author | Campanacci, DA | |
dc.contributor.author | Casanova, J | |
dc.contributor.author | Esler, C | |
dc.contributor.author | Ferrari, S | |
dc.contributor.author | Funovics, PT | |
dc.contributor.author | Gerrand, C | |
dc.contributor.author | Grimer, R | |
dc.contributor.author | Gronchi, A | |
dc.contributor.author | Haffner, N | |
dc.contributor.author | Hecker-Nolting, S | |
dc.contributor.author | Höller, S | |
dc.contributor.author | Jeys, L | |
dc.contributor.author | Jutte, P | |
dc.contributor.author | Leithner, A | |
dc.contributor.author | San-Julian, M | |
dc.contributor.author | Thorkildsen, J | |
dc.contributor.author | Vincenzi, B | |
dc.contributor.author | Windhager, R | |
dc.contributor.author | Whelan, J | |
dc.date.accessioned | 2016-05-12T10:10:36Z | |
dc.date.available | 2016-05-12T10:10:36Z | |
dc.date.issued | 2015-02 | |
dc.description.abstract | BACKGROUND: 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.citation | Eur J Cancer. 2015 Feb;51(3):374-81. | pt_PT |
dc.identifier.doi | 10.1016/j.ejca.2014.11.007 | pt_PT |
dc.identifier.uri | http://hdl.handle.net/10400.4/1921 | |
dc.language.iso | eng | pt_PT |
dc.peerreviewed | yes | pt_PT |
dc.subject | Neoplasias dos Ossos | pt_PT |
dc.subject | Condrossarcoma Mesenquimal | pt_PT |
dc.title | Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study | pt_PT |
dc.type | journal article | |
dspace.entity.type | Publication | |
rcaap.rights | openAccess | pt_PT |
rcaap.type | article | pt_PT |