The London Sarcoma Service, University College of London Hospital, London, United Kingdom; University Campus Bio-Medico, Rome, Italy.
Istituto Ortopedico Rizzoli di Bologna, Bologna, Italy.
Eur J Cancer. 2015 Feb;51(3):374-81. doi: 10.1016/j.ejca.2014.11.007. Epub 2014 Dec 16.
Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcoma with little evidence supporting treatment recommendations.
Specialist centres collaborated to report prognostic factors and outcome for 113 patients.
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).
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.
间叶性软骨肉瘤（MCS）是一种独特的、非常罕见的肉瘤，几乎没有证据支持治疗建议。
专家中心合作报告了 113 名患者的预后因素和结果。
中位年龄为 30 岁（范围：11-80），男女比例为 1.1。原发部位为四肢（40%）、躯干（47%）和头颈部（13%），41 例原发于软组织。17 例患者在诊断时已有转移。中位随访时间为 14.9 年（范围：1-34），总生存期（OS）中位数为 17 年（95%置信区间（CI）：10.3-28.6）。96 例局限性疾病患者中有 95 例接受了手术，54 例患者还接受了联合化疗。95 例患者中有 65 例存活，45 例无进展（5 例局部复发，34 例远处转移，11 例联合）。无进展生存期（PFS）和 OS 的中位数分别为 7（95%CI：3.03-10.96）和 20（95%CI：12.63-27.36）年。局限性疾病患者接受化疗与降低复发风险相关（P=0.046；风险比（HR）=0.482 95%CI：0.213-0.996）和死亡（P=0.004；HR=0.445 95%CI：0.256-0.774）。切缘清晰与局部复发频率较低相关（2%对 27%；P=0.002）。原发部位和起源对生存没有影响。诊断时无转移与更好的预后显著相关（P<0.0001）。关于放疗指征、剂量和分割的数据不够完整，无法对其对结果的影响进行评价。有转移的患者中位 OS 为 3 年（95%CI：0-4.25）。
MCS 的预后差异很大。诊断时的转移性疾病对生存的影响最大。完全切除和辅助化疗应被视为局限性疾病的标准治疗。
