Division of Pediatric Surgical Oncology, Department of Surgical Oncology, Tata Memorial Centre, Parel. 400012, Bombay, India.
J Pediatr Surg. 2013 Apr;48(4):764-70. doi: 10.1016/j.jpedsurg.2012.07.049.
The rarity of Ewing sarcoma of rib has resulted in paucity of data, particularly on the prognostic factors and pattern of relapses. We analyzed the recurrences in patients with primary nonmetastatic Ewing sarcoma of the rib and examined prognostic factors of poor outcome.
From January 2004 to January 2011, 37 patients were treated. After induction chemotherapy, complete (from costal cartilage to vertebra) or partial excision of involved rib with or without adjacent ribs was performed. Postoperative radiotherapy was administered for positive margins, poor response to chemotherapy, and large primary tumors with significant soft tissue component at presentation.
Disease relapsed in 16 patients: at the local site (n = 5), both local and distant (n = 2), and distant site only (n = 9). The projected 5-year cause-specific, relapse-free survival and local control were 50%, 44%, and 72%. Poor response to chemotherapy (>5% residual tumor) and resection of adjacent lung parenchyma (a surrogate for tumor extension) were adverse prognostic factors for relapse-free survival in multivariate analysis.
Relapses occurred more often at distant sites and had a poor outcome. In this study, poor histologic response to chemotherapy (P = .04) and the infiltration of adjacent lung parenchyma (P = .01) are adverse prognostic factors.
肋骨尤文肉瘤较为罕见，相关数据有限，特别是关于预后因素和复发模式的信息。我们分析了原发性非转移性肋骨尤文肉瘤患者的复发情况，并探讨了预后不良的相关因素。
2004 年 1 月至 2011 年 1 月，我们共收治了 37 例患者。诱导化疗后，我们对累及肋骨及相邻肋骨进行了完全（从肋软骨到椎体）或部分切除，对于切缘阳性、化疗反应不佳以及原发肿瘤较大且伴有明显软组织受累的患者，术后给予放疗。
16 例患者发生疾病复发：局部复发 5 例，局部和远处转移 2 例，远处转移 9 例。预计 5 年的特异性无病生存率、无复发生存率和局部控制率分别为 50%、44%和 72%。多因素分析显示，化疗反应不良（>5%残留肿瘤）和切除相邻肺实质（肿瘤扩展的替代指标）是无复发生存率的不良预后因素。
复发更常发生于远处部位，且预后较差。在本研究中，化疗后组织学反应不良（P =.04）和相邻肺实质浸润（P =.01）是不良预后因素。
