The Soft Tissue Sarcoma Committee of the Children's Oncology Group, Monrovia, CA; Aaron R. Weiss, Maine Medical Center, Portland, ME; Elizabeth R. Lyden and James R. Anderson, University of Nebraska Medical Center, Omaha, NE; Douglas S. Hawkins, Seattle Children's Hospital, Fred Hutchinson Cancer Research Center, University of Washington, Seattle, WA; Sheri L. Spunt, St. Jude Children's Research Hospital and the University of Tennessee Health Science Center, Memphis, TN; David O. Walterhouse, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL; Suzanne L. Wolden, Memorial Sloan-Kettering Cancer Center, New York, NY; David M. Parham, University of Oklahoma Health Sciences Center, Oklahoma City, OK; David A. Rodeberg, Children's Hospital of Pittsburgh, Pittsburgh, PA; Simon C. Kao, University of Iowa Hospitals and Clinics, Iowa City, IA; and Richard B. Womer, The Children's Hospital of Philadelphia, Philadelphia, PA.
J Clin Oncol. 2013 Sep 10;31(26):3226-32. doi: 10.1200/JCO.2012.44.6476. Epub 2013 Aug 12.
To simplify the recommended staging evaluation by correlating tumor and clinical features with patterns of distant metastasis in newly diagnosed patients with embryonal rhabdomyosarcoma (ERMS) or alveolar rhabdomyosarcoma (ARMS).
Patient data from the Intergroup Rhabdomyosarcoma Study Group and the Children's Oncology Group over two periods were analyzed: 1991 to 1997 and 1999 to 2004. We used recursive partitioning analyses to identify factors (including histology, age, regional nodal and distant metastatic status, tumor size, local invasiveness, and primary site) that divided patients into subsets with the most different rates of metastatic disease.
Of the 1,687 patients analyzed, 5.7% had lung metastases, 4.8% had bone involvement, and 6% had bone marrow (BM) involvement. Rhabdomyosarcoma (RMS) without local invasion (T1) had a low rate of metastasis for all distant sites, especially ERMS (0% bone, 0% BM). ARMS with local invasion (T2) had a higher rate of metastasis for all distant sites (13% lung, 18% bone, 23% BM). ERMS, T2 also had a higher rate of metastatic lung involvement (9%). The likelihood of bone or BM involvement increased in the presence of lung metastases (41% with, 6% without). Regional nodal metastases (N1) predicted a high rate of metastasis in all distant sites (14% lung, 14% bone, 18% BM). A staging algorithm was developed.
Staging studies in childhood RMS can be tailored to patients' presenting characteristics. Bone marrow aspirate and biopsy and bone scan are unnecessary in at least one third of patients with RMS.
通过将肿瘤和临床特征与新诊断的胚胎性横纹肌肉瘤（ERMS）或肺泡横纹肌肉瘤（ARMS）患者的远处转移模式相关联，简化推荐的分期评估。
分析了两个时期（1991 年至 1997 年和 1999 年至 2004 年）来自横纹肌肉瘤研究组和儿童肿瘤组的患者数据。我们使用递归分区分析来确定因素（包括组织学、年龄、区域淋巴结和远处转移状态、肿瘤大小、局部侵袭性和原发部位），这些因素将患者分为转移疾病发生率差异最大的亚组。
在分析的 1687 名患者中，5.7%有肺部转移，4.8%有骨骼侵犯，6%有骨髓（BM）侵犯。无局部侵犯（T1）的横纹肌肉瘤（RMS）在所有远处部位的转移率都较低，尤其是 ERMS（无骨转移，无 BM 转移）。有局部侵犯（T2）的 ARMS 在所有远处部位的转移率都较高（肺部转移率为 13%，骨骼转移率为 18%，BM 转移率为 23%）。T2 的 ERMS 也有较高的肺部转移率（9%）。存在肺部转移时，发生骨骼或 BM 侵犯的可能性增加（有转移者为 41%，无转移者为 6%）。区域淋巴结转移（N1）预测所有远处部位的转移率都很高（肺部转移率为 14%，骨骼转移率为 14%，BM 转移率为 18%）。开发了一种分期算法。
儿童 RMS 的分期研究可以根据患者的表现特征进行调整。至少三分之一的 RMS 患者不需要进行骨髓抽吸和活检以及骨扫描。
