Analysis of prognostic factors in patients with nonmetastatic rhabdomyosarcoma treated on intergroup rhabdomyosarcoma studies III and IV: The Children's Oncology Group
Analysis of prognostic factors in patients with nonmetastatic rhabdomyosarcoma treated on intergroup rhabdomyosarcoma studies III and IV: The Children's Oncology Group
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DOI：
10.1200/jco.2005.05.3801
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发表时间：
2006-08-20
影响因子：
45.3
通讯作者：
Meyer, William H.
中科院分区：
文献类型：
--
作者：
Meza, Jane L.;Anderson, James;Meyer, William H.
局限性横纹肌肉瘤（RMS）或未分化肉瘤（UDS）的预后受年龄、组织学、原发解剖部位、疾病范围患者和方法我们评估了患者和疾病特征预测非转移性RMS或UDS患者在组间横纹肌肉瘤研究（IRS）-III中治疗的结果的能力结果胚胎型RMS（embryonal RMS，ERMS）1期I组或IIa组、2期I组或III组患者的5年无失败生存率（FFS）估计为90%。估计5年FFS率为87%的患者为ERMS 1期，IIb或IIc组; 1期，III组非眼眶; 2期，II组; 3期，I或II组;和73%的患者为ERMS 2或3期，III组。年龄小于1岁或10岁或10岁以上的2期或3期III组ERMS伴浸润性（T2）肿瘤患者（56%）和2期或3期III组肢体原发性肿瘤患者（43%）的估计5年FFS率较差。总体而言，牙槽骨RMS（ARMS）或UDS患者的结局比ERMS患者差。然而，对于I或II组（80%）或III组（76%）疾病的有利部位ARMS/UDS患者，5年FFS率良好。在I或II组（66%）或III组（45%）疾病的不利部位，ARMS/UDS患者的FFS率较差。估计5年的FFS率为31%，第三组ARMS/UDS患者在不利的网站与区域淋巴结疾病，这是类似的转移性RMS.Conclusion患者和疾病特征识别不同的子集与不同的结果，允许软组织肉瘤委员会的儿童肿瘤组，以完善风险适应性治疗分配。
Purpose The outcome for localized rhabdomyosarcoma (RMS) or undifferentiated sarcoma (UDS) is affected by age, histology, primary anatomic site, extent of disease, and therapy.Patients and Methods We evaluated patient and disease characteristics for their ability to predict outcome for patients with nonmetastatic RMS or UDS treated on Intergroup Rhabdomyosarcoma Study (IRS)-III (1984 to 1991) or IRS-IV (1991 to 1997).Results The estimated 5-year failure-free survival (FFS) rate was 90% for patients with embryonal RMS (ERMS) stage 1, group I or IIa; stage 2, group I; or group III orbit. The estimated 5-year FFS rate was 87% for patients with ERMS stage 1, group IIb or IIc; stage 1, group III nonorbit; stage 2, group II; and stage 3, group I or II; and 73% for patients with ERMS stage 2 or 3, group III. The estimated 5-year FFS rate was poor for patients with stage 2 or 3, group III ERMS with invasive (T2) tumors who were age younger than 1 year or 10 years or older (56%) and patients with stage 2 or 3, group III extremity primary tumors (43%). Overall, outcomes for patients with alveolar RMS (ARMS) or UDS were worse than for patients with ERMS. However, the 5-year FFS rate was good for patients with ARMS/UDS at favorable sites with group I or II (80%) or group III (76%) disease. The FFS rate was poorer for patients with ARMS/UDS at unfavorable sites with group I or II (66%) or group III (45%) disease. The estimated 5-year FFS rate was 31% for patients with group III ARMS/UDS at unfavorable sites with regional lymph node disease, which is similar to metastatic RMS.Conclusion Patient and disease characteristics identify distinct subsets with different outcomes, allowing the Soft Tissue Sarcoma Committee of the Children's Oncology Group to refine risk-adapted therapy assignment.