Neville H L, Andrassy R J, Lobe T E, Bagwell C E, Anderson J R, Womer R B, Crist W M, Wiener E S
IRS Group of the Children's Cancer Group and the Pediatric Oncology Group, Rochester, MN 55905-0001, USA.
J Pediatr Surg. 2000 Feb;35(2):317-21. doi: 10.1016/s0022-3468(00)90031-9.
During the fourth Intergroup Rhabdomyosarcoma (RMS) Study (IRS IV, 1991-97), a preoperative staging system was evaluated prospectively for the first time. The authors evaluated this staging system and the role of surgery in extremity RMS in contemporary multimodal therapy.
A total of 139 patients (71 girls; median age, 6 years) were entered in IRS IV with extremity-site RMS. Stage was assigned by the IRSG Preoperative Staging System. Postsurgical group was determined by tumor status after initial surgical intervention. Multivariate analysis was performed using all pretreatment factors that were significant by univariate analysis, including clinical Group (i.e., I through IV), tumor invasiveness (T1,T2), nodal status (N0,N1), and tumor size (< or > or =5 cm). Failure-free survival rates (FFS) and survival rates were estimated using the Kaplan and Meier method.
Preoperative staging and clinical group distribution were as follows: Stage 2, n = 34; Stage 3, n = 73; Stage 4, n = 32; Group I, n = 31; Group II, n = 21; Group III, n = 54; Group IV, n = 33. Three-year FFS was 55%, and the overall survival rate was 70%. Eighty-seven patients had either unresectable, gross residual disease (Group III) or metastases (Group IV). FFS was significantly worse for these patients with advanced disease, compared with that for patients with complete resection or with only microscopic residual tumor (i.e., Group I or II; Group I, 3-year FFS, 91%; Group II, 72%; Group III, 50%; Group IV, 23%; P<.001). Lymph nodes were evaluated surgically in 76 patients with positive results in 38. Clinically, 13 additional patients had nodal disease. Both stage and group were highly predictive of outcome and were highly correlated. By multivariate analysis, none of the other variables were predictors of FFS.
This review confirms the utility of pretreatment staging for stratification of patients with extremity RMS with widely different risks of relapse, thereby paving the way for development of risk-based therapy. Group (operative staging) remains the most important predictor of FFS, emphasizing the importance of complete gross resection at initial surgical intervention, when feasible without loss of limb function. The high incidence of nodal disease in the patients who had lymph node biopsy confirms the need for surgical evaluation of lymph nodes to ensure accurate staging in children with extremity rhabdomyosarcoma.
在第四届横纹肌肉瘤（RMS）多组协作研究（IRS IV，1991 - 1997年）期间，首次对一种术前分期系统进行了前瞻性评估。作者评估了该分期系统以及手术在当代多模式治疗肢体RMS中的作用。
共有139例患者（71例女孩；中位年龄6岁）因肢体部位RMS纳入IRS IV研究。分期由IRSG术前分期系统确定。术后分组根据初次手术干预后的肿瘤状态确定。使用单因素分析中有显著意义的所有预处理因素进行多因素分析，包括临床分组（即I至IV组）、肿瘤侵袭性（T1、T2）、淋巴结状态（N0、N1）和肿瘤大小（<或>或=5 cm）。采用Kaplan - Meier法估计无病生存率（FFS）和生存率。
术前分期和临床分组分布如下：2期，n = 34；3期，n = 73；4期，n = 32；I组，n = 31；II组，n = 21；III组，n = 54；IV组，n = 33。三年FFS为55%，总生存率为70%。87例患者存在不可切除的大体残留病灶（III组）或转移（IV组）。与完全切除或仅存在微小残留肿瘤的患者（即I组或II组；I组，三年FFS，91%；II组，72%；III组，50%；IV组，23%；P<0.001）相比，这些晚期疾病患者的FFS明显更差。76例患者接受了淋巴结手术评估，其中38例结果为阳性。临床上，另有13例患者存在淋巴结疾病。分期和分组均对预后具有高度预测性且高度相关。通过多因素分析，其他变量均不是FFS的预测因素。
本综述证实了术前分期对于分层具有广泛不同复发风险的肢体RMS患者的实用性，从而为基于风险的治疗发展铺平了道路。分组（手术分期）仍然是FFS的最重要预测因素，强调了在可行且不丧失肢体功能的情况下，初次手术干预时进行完全大体切除的重要性。接受淋巴结活检的患者中淋巴结疾病的高发生率证实了对肢体横纹肌肉瘤患儿进行淋巴结手术评估以确保准确分期的必要性。
