Andrassy R J, Wiener E S, Raney R B, Lawrence W, Lobe T E, Corpron C A, Maurer H M
Intergroup Rhabdomyosarcoma Study Committee.
Ann Surg. 1998 Feb;227(2):170-3. doi: 10.1097/00000658-199802000-00003.
This study reviews the experience of the Intergroup Rhabdomyosarcoma Studies (IRS) II and III with thoracic sarcomas.
Thoracic sarcomas in children present special problems with histologic diagnosis, total resection of tumor, and local control. Previous studies have shown high rates of local and distant relapse.
Data from evaluable patients enrolled in IRS II and IRS III were reviewed. Progression-free and overall survival were estimated by the Kaplan-Meier method and statistical differences were calculated by the log-rank test. The multivariate regression analysis of data was performed using the Cox proportional hazards model.
A total of 84 patients presented with thoracic sarcomas during IRS II (51 patients) and IRS III (33 patients). Of these, 76 were chest wall tumors, 3 were lung tumors, 4 were pleural tumors, and 1 arose from the heart. The average age at diagnosis was 8.5 years (range, 1 month-20.5 years). Thirty-three patients had alveolar histology, 17 had embryonal histology, and 12 had extraosseous Ewing's sarcoma, with indeterminate, undifferentiated, and pleomorphic histologies in the remaining 22 patients. Thirteen patients presented as group I (localized disease completely resected), 18 as group II (microscopic residual or nodal disease), 31 as group III (gross residual disease or biopsy only), and 22 as group IV (distant metastatic disease). Sixty patients (71%) achieved a complete response. Thirty-nine patients had a local relapse, and 22 had a distant relapse. Thirty-five patients (42%) survived with a mean follow-up of 1.8 years (range 0.7-11.7 years); 49 (58%) have died with an average survival of 1.1 years (range 0.3-11.7 years). Four patients (5%) died of infection or treatment complications. Progression-free survival was not significantly associated with histology, site, clinical group, or IRS study. Overall survival was significantly associated with clinical group, size, and local or distant recurrence, but not with histology or IRS study by univariate analysis. In a multivariate analysis, only clinical group and local and distant recurrence showed statistical significance (p < 0.002, < 0.006, and < 0.001, respectively).
As expected, outcome was better for patients with totally resected or microscopically residual tumor after resection. A higher rate of recurrence in group I versus group II,although not statistically significant, suggests that identification of margin status may be difficult, and these patients may require more intensive efforts for local control. These patients continued to have a high rate of local and distant relapse, and new strategies for preventing these are under development.
本研究回顾了横纹肌肉瘤协作组（IRS）II和III期研究中胸段肉瘤的治疗经验。
儿童胸段肉瘤在组织学诊断、肿瘤全切及局部控制方面存在特殊问题。既往研究显示局部和远处复发率较高。
回顾IRS II期和III期研究中可评估患者的数据。采用Kaplan-Meier法估计无进展生存期和总生存期，并通过对数秩检验计算统计学差异。使用Cox比例风险模型对数据进行多因素回归分析。
IRS II期（51例患者）和IRS III期（33例患者）共有84例患者表现为胸段肉瘤。其中，76例为胸壁肿瘤，3例为肺肿瘤，4例为胸膜肿瘤，1例起源于心脏。诊断时的平均年龄为8.5岁（范围1个月至20.5岁）。33例患者为肺泡型组织学，17例为胚胎型组织学，12例为骨外尤文肉瘤，其余22例患者组织学类型为不确定、未分化和多形性。13例患者为I组（局限性疾病完全切除），18例为II组（镜下残留或淋巴结疾病），31例为III组（大体残留疾病或仅活检），22例为IV组（远处转移疾病）。60例患者（71%）获得完全缓解。39例患者出现局部复发，22例出现远处复发。35例患者（42%）存活，平均随访1.8年（范围0.7至11.7年）；49例（58%）死亡，平均生存期1.1年（范围0.3至11.7年）。4例患者（5%）死于感染或治疗并发症。无进展生存期与组织学类型、部位、临床分组或IRS研究无显著相关性。单因素分析显示，总生存期与临床分组、肿瘤大小及局部或远处复发显著相关，但与组织学类型或IRS研究无关。多因素分析显示，仅临床分组及局部和远处复发具有统计学意义（p分别<0.002、<0.006和<0.001）。
正如预期的那样，肿瘤完全切除或切除后镜下残留的患者预后较好。I组与II组相比复发率较高，尽管无统计学意义，提示切缘状态的判定可能存在困难，这些患者可能需要更积极的局部控制措施。这些患者局部和远处复发率仍然较高，预防复发的新策略正在研发中。
