Andrassy R J, Corpron C A, Hays D, Raney R B, Wiener E S, Lawrence W, Lobe T E, Bagwell C, Maurer H M
J Pediatr Surg. 1996 Jan;31(1):191-6. doi: 10.1016/s0022-3468(96)90346-2.
Prognostic factors for extremity sarcomas have been reported previously, after analysis of Intergroup Rhabdomyosarcomas Studies (IRS) I and II. This report reviews the experience of IRS III (1984-1992), in light of these reported factors, and the pretreatment factors used in the staging system currently being evaluated in IRS IV. The results of treatment of extremity sarcomas in IRS III are reported.
The charts of all patients entered in IRS III with an extremity-site tumor were reviewed. This group included patients with shoulder girdle and buttock sites. All patients were treated according to IRS III protocols. Survival rates were estimated by the method of Kaplan and Meier, and comparisons among groups of patients were made using a log-rank test. A multivariate analysis was performed to analyze all pretreatment factors that were significant by univariate analysis.
Of the 189 patients entered in IRS III with extremity sites, 88 (47%) had the most common alveolar histology. Fifty-nine patients were in group I at the time of presentation (completely resected disease), 48 in group II (microscopic residual disease), 36 in group III (gross residual disease), and 46 in group IV (metastatic disease). By univariate analysis, the significant prognostic factors affecting survival were clinical group, age at time of diagnosis, tumor size, distant metastases, nodal metastases, and local and distant recurrence. By multivariate analysis of pretreatment factors, age at time of diagnosis, nodal metastases, and distant metastases were significant prognostic factors affecting survival; tumor size approached significance. Both the clinical group system and pretreatment staging system (Lawrence/Gehan) predicted significant differences in survival between groups of patients. The lower survival rate among group II and III node-negative patients with a distal tumor (who, with more extensive surgery, could have been in group I) in comparison to group I patients with a distal lesion, approached significance. In patients without distant metastases, survival was significantly different in those patients that had negative nodes from those in whom nodes were not biopsied.
This review confirms that both clinical grouping and the new pretreatment staging system used in IRS IV can predict the likelihood of survival of children with extremity sarcomas. By multivariate analysis of the elements included in the staging system, nodal metastases, distant metastases, and tumor size were useful in predicting survival. In addition, age was a significant predictor. This study confirms previous suggestions that complete excision with gross and microscopically negative margins is preferable in the treatment of children with extremity rhabdomyosarcomas.
在对横纹肌肉瘤协作组研究（IRS）I和II进行分析之后，先前已有关于肢体肉瘤预后因素的报道。本报告根据这些已报道的因素以及IRS IV目前正在评估的分期系统中所使用的预处理因素，回顾了IRS III（1984 - 1992年）的经验。报告了IRS III中肢体肉瘤的治疗结果。
回顾了IRS III中所有患有肢体部位肿瘤患者的病历。该组包括肩带和臀部部位的患者。所有患者均按照IRS III方案进行治疗。采用Kaplan - Meier法估计生存率，并使用对数秩检验对患者组之间进行比较。进行多变量分析以分析单变量分析中有显著意义的所有预处理因素。
在IRS III中登记的189例肢体部位患者中，88例（47%）具有最常见的肺泡组织学类型。59例患者在就诊时属于I组（疾病完全切除），48例属于II组（镜下残留疾病），36例属于III组（肉眼残留疾病），46例属于IV组（转移性疾病）。通过单变量分析，影响生存的显著预后因素为临床分组、诊断时年龄、肿瘤大小、远处转移、淋巴结转移以及局部和远处复发。通过对预处理因素的多变量分析，诊断时年龄、淋巴结转移和远处转移是影响生存的显著预后因素；肿瘤大小接近显著水平。临床分组系统和预处理分期系统（Lawrence/Gehan）均预测患者组之间的生存存在显著差异。与I组远端病变患者相比，II组和III组远端肿瘤且淋巴结阴性的患者（若进行更广泛的手术本可属于I组）生存率较低，接近显著水平。在无远处转移的患者中，有阴性淋巴结的患者与未进行淋巴结活检的患者生存率存在显著差异。
本综述证实，临床分组以及IRS IV中使用的新预处理分期系统均可预测肢体肉瘤患儿的生存可能性。通过对分期系统中各项因素的多变量分析，淋巴结转移、远处转移和肿瘤大小对预测生存有用。此外，年龄也是一个显著的预测因素。本研究证实了先前的建议，即在治疗肢体横纹肌肉瘤患儿时，采用肉眼和镜下切缘阴性的完全切除更为可取。
