Casanova Michela, Meazza Cristina, Gronchi Alessandro, Fiore Marco, Zaffignani Elena, Podda Marta, Collini Paola, Gandola Lorenza, Ferrari Andrea
Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Via G. Venezian, 1, 20133, Milan MI, Italy.
J Child Orthop. 2007 Sep;1(3):195-203. doi: 10.1007/s11832-007-0042-4. Epub 2007 Sep 1.
The extremity site is a peculiar location for soft-tissue sarcomas (STS) of children and adolescents. Here we report the experience of the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, Italy, concerning 204 patients with STS of the limbs treated between 1977 and 2006.
The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma. Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy.
For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure. The outcome was particularly poor for patients with large invasive tumors, hand/foot involvement and/or nodal or distant metastases, and for patients who were not given radiotherapy. For the extraosseous Ewing sarcoma cases, 5-year EFS was 74%. For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors.
While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results. The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery.
四肢部位是儿童和青少年软组织肉瘤（STS）的特殊发病部位。在此，我们报告意大利米兰国立肿瘤研究所儿科肿瘤病房对1977年至2006年间收治的204例四肢STS患者的治疗经验。
该研究系列包括52例横纹肌肉瘤（RMS）患者（其中65%为肺泡型亚型）、9例骨外尤文肉瘤患者和143例非横纹肌肉瘤软组织肉瘤（NRSTS）患者，其中38%为滑膜肉瘤。患者接受了包括手术、化疗和放疗在内的多模式治疗。
对于RMS患者，5年无事件生存率（EFS）为37.1%，远处转移是治疗失败的主要原因。对于侵袭性大的肿瘤、累及手/足和/或有淋巴结或远处转移的患者，以及未接受放疗的患者，预后尤其差。对于骨外尤文肉瘤病例，5年EFS为74%。对于NRSTS病例，5年EFS为72.6%：肿瘤大小和局部侵袭性、肿瘤分级、恶性外周神经鞘瘤（MPNST）组织学类型和远处转移是主要的预后因素。
虽然四肢是NRSTS最常见的发病部位，且其预后通常比躯干肿瘤更有利，但四肢RMS的临床特征往往与其他部位的RMS不同，不良预后因素（如肺泡型亚型）的发生率更高，因此治疗效果不理想。这些患者的治疗复杂，必然需要多学科协作，不仅需要在临床试验中治疗儿童和青少年的足够经验，还需要整形外科领域的特殊技能。
