Pediatric Onco-Hematology and Stem Cell Transplant and Cellular Therapy, Regina Margherita Children's Hospital, Turin, Italy.
Eur J Cancer. 2013 Nov;49(17):3658-65. doi: 10.1016/j.ejca.2013.06.045. Epub 2013 Jul 24.
The aim of this study was to describe the Italian Association of Pediatric Hematology and Oncology (AIEOP) and Italian Sarcoma Group (ISG) experience from 1980 to 2009 on 112 patients with Ewing sarcoma (ES) occurring in unusual sites such as the craniofacial bones (CF), hands or feet (HF), or the mobile spine. These sites were grouped because their rarity as ES localisations.
Twenty-six patients had CF ES (23%), 37 patients had HF ES (33%) and 49 patients had mobile spine ES (44%). A total of 26 patients presented with synchronous metastatic disease (23%). The local treatment with surgery and/or radiotherapy differed among ES sites. Systemic therapy was administrated according to the protocols in use over the years.
From the data available, the histological/radiological response was higher for HF-patients even not statistical significant (good responders: CF 41%, HF 65% and mobile spine 39%, P = 0.NS) and the probability of achieving complete response was similar among the three sites (CF 87%, HF 83% and spine 74%, P = 0.44). Ten year overall survival (OS) was 61% (95% confidence interval [CI] 39-82), 63% (95% CI 37-89) and 64% (95% CI 49-79) for CF, HF or vertebral ES, respectively (P = NS). Ten year OS for non-metastatic patients was 60% (95% CI 36-83), 75% (95% CI 56-94) and 67% (95% CI 47-89) for CF, HF and mobile spine patients respectively (P = NS). Ten year OS was 45% (95% CI, 31-84) and 70% (95% CI, 61-85, [p = 0.01]) for metastatic and localised ES, respectively.
The probability of successful treatment did not differ from ES of the extremities. Furthermore, our series confirm the poor prognosis for patients with metastatic disease. Our data do not strengthen the need for a specific protocol for unusual site ES.
本研究的目的是描述意大利儿科血液学和肿瘤学协会（AIEOP）和意大利肉瘤组（ISG）从 1980 年到 2009 年对 112 例发生在颅面骨（CF）、手或脚（HF）或活动脊柱等不常见部位的尤文肉瘤（ES）患者的经验。这些部位被归为一组，因为它们是 ES 局部定位的罕见部位。
26 例患者为颅面骨 ES（23%），37 例患者为 HF-ES（33%），49 例患者为活动脊柱 ES（44%）。共有 26 例患者出现同步转移性疾病（23%）。手术和/或放疗的局部治疗因 ES 部位而异。根据多年来使用的方案进行系统治疗。
根据现有数据，HF 患者的组织学/影像学反应更高，尽管没有统计学意义（反应良好者：CF41%、HF65%和活动脊柱 39%，P=0.NS），并且三个部位的完全缓解率相似（CF87%、HF83%和脊柱 74%，P=0.44）。CF、HF 或脊柱 ES 的 10 年总生存率（OS）分别为 61%（95%置信区间 [CI] 39-82%）、63%（95% CI 37-89%）和 64%（95% CI 49-79%）（P=NS）。无转移患者的 10 年 OS 分别为 CF、HF 和活动脊柱患者的 60%（95% CI 36-83%）、75%（95% CI 56-94%）和 67%（95% CI 47-89%）（P=NS）。转移和局部 ES 的 10 年 OS 分别为 45%（95% CI，31-84%）和 70%（95% CI，61-85%，[p=0.01]）。
成功治疗的可能性与四肢 ES 没有区别。此外，我们的系列研究证实了转移性疾病患者预后不良。我们的数据并没有加强对不常见部位 ES 制定特定方案的必要性。
