Mesenchymal chondrosarcoma: Prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study. Issue 3 (February 2015)

• Record Type: Journal Article
• Authors: Frezza, Anna Maria; Cesari, Marilena; Baumhoer, Daniel; Biau, David; Bielack, Stephen; Campanacci, Domenico Andrea; Casanova, José; Esler, Claire; Ferrari, Stefano; Funovics, Philipp T.; Gerrand, Craig; Grimer, Robert; Gronchi, Alessandro; Haffner, Nicolas; Hecker-Nolting, Stefanie; Höller, Sylvia; Jeys, Lee; Jutte, Paul; Leithner, Andreas; San-Julian, Mikel; Thorkildsen, Joachim; Vincenzi, Bruno; Windhager, Reinhard; Whelan, Jeremy
• Is Part Of: European journal of cancer. Volume 51:Issue 3(2015:Feb.)
• Page Start: 374
• Page End: 381
• Publication Date: 2015-02
• Subjects: Cancer -- Periodicals; Neoplasms -- Periodicals; Cancer -- Périodiques; Cancer; Tumors; Electronic journals; Periodicals; Electronic journals; 616.994
• DOI: 10.1016/j.ejca.2014.11.007
• Languages: English
• ISSNs: 0959-8049

Abstract

<abstract xml:lang="en" abstract-type="author" id="ab005"> <title id="st080">Abstract</title> <sec> <title id="st085">Background</title> <p id="sp0005">Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcoma with little evidence supporting treatment recommendations.</p> </sec> <sec> <title id="st090">Patients and methods</title> <p id="sp0010">Specialist centres collaborated to report prognostic factors and outcome for 113 patients.</p> </sec> <sec> <title id="st095">Results</title> <p id="sp0015">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 (<italic>P</italic> = 0.046; hazard ratio (HR) = 0.482 95% CI: 0.213–0.996) and death (<italic>P</italic> = 0.004; HR = 0.445 95% CI: 0.256–0.774). Clear resection margins predicted less frequent local recurrence (2% versus 27%; <italic>P</italic> = 0.002). Primary site and origin did not influence survival. The absence of metastases at diagnosis was associated with a significantly better outcome (<italic>P</italic> < 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).</p> </sec> <sec> <title id="st100">Conclusions</title> <p id="sp0020">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.</p> </sec> </abstract>