AbstractBackground:Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcomawith little evidence supporting treatment recommendations.Patients and methods:Specialist centres collaborated to report prognostic factors and out-come for 113 patients.Results:Median age was 30 years (range: 11–80), male/female ratio 1.1. Primary sites wereextremities (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 receivedcombination chemotherapy. Sixty-five of 95 patients are alive and 45 progression-free (5 localrecurrence, 34 distant metastases, 11 combined). Median progression-free survival (PFS) andOS were 7 (95% CI: 3.03–10.96) and 20 (95% CI: 12.63–27.36) years respectively. Chemother-apy administration in patients with localised disease was associated with reduced risk of recur-rence (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 recur-rence (2% versus 27%;P= 0.002). Primary site and origin did not influence survival. Theabsence of metastases at diagnosis was associated with a significantly better outcome(P< 0.0001). Data on radiotherapy indications, dose and fractionation were insufficientlycomplete, to allow comment of its impact on outcomes. Median OS for patients with metas-tases at presentation was 3 years (95% CI: 0–4.25).Conclusions:Prognosis in MCS varies considerably. Metastatic disease at diagnosis has thestrongest impact on survival. Complete resection and adjuvant chemotherapy should be con-sidered as standard of care for localised disease

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

Vincenzi B;
2015-01-01

Abstract

AbstractBackground:Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcomawith little evidence supporting treatment recommendations.Patients and methods:Specialist centres collaborated to report prognostic factors and out-come for 113 patients.Results:Median age was 30 years (range: 11–80), male/female ratio 1.1. Primary sites wereextremities (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 receivedcombination chemotherapy. Sixty-five of 95 patients are alive and 45 progression-free (5 localrecurrence, 34 distant metastases, 11 combined). Median progression-free survival (PFS) andOS were 7 (95% CI: 3.03–10.96) and 20 (95% CI: 12.63–27.36) years respectively. Chemother-apy administration in patients with localised disease was associated with reduced risk of recur-rence (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 recur-rence (2% versus 27%;P= 0.002). Primary site and origin did not influence survival. Theabsence of metastases at diagnosis was associated with a significantly better outcome(P< 0.0001). Data on radiotherapy indications, dose and fractionation were insufficientlycomplete, to allow comment of its impact on outcomes. Median OS for patients with metas-tases at presentation was 3 years (95% CI: 0–4.25).Conclusions:Prognosis in MCS varies considerably. Metastatic disease at diagnosis has thestrongest impact on survival. Complete resection and adjuvant chemotherapy should be con-sidered as standard of care for localised disease
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/20.500.12610/10914
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