Ferrari Andrea, Bisogno Gianni, Alaggio Rita, Cecchetto Giovanni, Collini Paola, Rosolen Angelo, Meazza Cristina, Indolfi Paolo, Garaventa Alberto, De Sio Luigi, D'Angelo Paolo, Tamaro Paolo, Casanova Michela, Carli Modesto
Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Via G. Venezian 1, Milan, Italy.
Eur J Cancer. 2008 Jun;44(9):1202-9. doi: 10.1016/j.ejca.2008.03.016. Epub 2008 Apr 25.
The outcome of patients with non-extremity synovial sarcoma (SS) is generally worse than that of patients with limb tumours.
The present study analysed a series of 115 consecutive SS patients treated in Italian paediatric protocols (period 1979-2005), mainly focusing on the 30 cases arising from 'axial' sites (16 head-neck, 8 trunk, 4 lung-pleura and 2 retroperitoneum).
Initial gross resection was achieved in 40% of axial cases and in 80% of limb SS (p<0.0001). Five-year EFS and overall survival (OS) were, respectively, 43.3% and 55.1% for axial SS, and 69.6% (p=0.0068) and 84.0% (p=0.0004) for extremity SS. Local progression/recurrence was the cause of treatment failure in 75% of relapsing patients axial disease.
Our findings emphasise that children and adolescents with SS originating at non-extremity locations have a worse prognosis than those with limb SS. Tumour site should be considered when defining a risk-adapted treatment strategy for SS.
非四肢滑膜肉瘤（SS）患者的预后通常比肢体肿瘤患者差。
本研究分析了115例按照意大利儿科方案治疗的连续性SS患者（1979 - 2005年期间），主要聚焦于30例起源于“轴位”部位的病例（16例头颈部、8例躯干、4例肺 - 胸膜和2例腹膜后）。
40%的轴位病例和80%的肢体SS病例实现了初次根治性切除（p<0.0001）。轴位SS的5年无事件生存率（EFS）和总生存率（OS）分别为43.3%和55.1%，肢体SS分别为69.6%（p = 0.0068）和84.0%（p = 0.0004）。局部进展/复发是75%复发的轴位疾病患者治疗失败的原因。
我们的研究结果强调，起源于非四肢部位的SS儿童和青少年的预后比肢体SS患者差。在为SS制定风险适应性治疗策略时应考虑肿瘤部位。
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