Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study.
Eur J Cancer
; 51(3): 374-81, 2015 Feb.
Article
em En
| MEDLINE
| ID: mdl-25529371
ABSTRACT
BACKGROUND:
Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcoma with little evidence supporting treatment recommendations. PATIENTS ANDMETHODS:
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.Palavras-chave
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Base de dados:
MEDLINE
Assunto principal:
Neoplasias Ósseas
/
Condrossarcoma Mesenquimal
Idioma:
En
Ano de publicação:
2015
Tipo de documento:
Article