Background Preoperative chemotherapy plays an important role in the surgical management of unresectable neuroblastoma. Its response to chemotherapy has been variable due to the tumor’s heterogeneity. We aimed to evaluate the effects of preoperative chemotherapy on MYCN-amplified (MYCNA) neuroblastoma that would impact on surgical resection. Methods Patients with MYCNA neuroblastoma who received preoperative chemotherapy followed by surgical resection performed at our center were included. The tools of response evaluated included tumor volume reduction (TVR), reduction in image-defined risk factors (IDRFs), percentage tumor necrosis (Nec), and surgical complications. Results Among 62 patients evaluated, mean age was 3.0 (range, 0.9–11.8) years, and primary tumors were distributed in the abdomen (n=59), pelvis (n=2), and thorax (n=1). The patients were in stages L2 (n=14) and M (n=48). Surgery was performed after median of 4 (range, 2–10) cycles of chemotherapy. On completion of preoperative chemotherapy, 41 (66.1%) patients had TVR >65%, 24 (42.9%) responded with reduced IDRFs, 47 (75.8%) tumors had Nec >50%, and 27 patients suffered 31 surgical complications. Majority (83.9%) continued to have IDRFs at surgery. IDRFs commonly encountered were encasement of renal pedicles (n=50), superior mesenteric artery (n=46), celiac axis (n=45), and aorta/vena cava (n=44), and most remained refractory to resolution. Patients with TVR >65% were associated with Nec >50% (87.5% vs 54.5%, p=0.004) and reduced IDRFs (46.3% vs 19%, p=0.035), but not with the incidence of surgical complications. Conclusions Majority of MYCNA neuroblastomas were highly chemosensitive as they experienced high TVR, reduced IDRFs, and high Nec, and hence created favorable conditions for surgical resection. Poor responders and persistent IDRFs that were commonly refractory to preoperative chemotherapy remained a surgical challenge.

中文翻译：

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术前化疗对伴有 MYCN 扩增的神经母细胞瘤的影响：外科医生的观点

背景 术前化疗在不可切除的神经母细胞瘤的手术治疗中起着重要作用。由于肿瘤的异质性，它对化疗的反应是可变的。我们的目的是评估术前化疗对 MYCN 扩增 (MYCNA) 神经母细胞瘤的影响，这将影响手术切除。方法 纳入在我中心接受术前化疗后手术切除的 MYCNA 神经母细胞瘤患者。评估的反应工具包括肿瘤体积缩小 (TVR)、图像定义的风险因素 (IDRF) 减少、肿瘤坏死百分比 (Nec) 和手术并发症。结果 在接受评估的 62 名患者中，平均年龄为 3.0（范围，0.9-11.8）岁，原发肿瘤分布在腹部（n=59）、骨盆（n=2）和胸部（n=1）。患者处于 L2 (n=14) 和 M (n=48) 阶段。手术是在中位 4（范围，2-10）个化疗周期后进行的。完成术前化疗后，41 名（66.1%）患者的 TVR >65%，24 名（42.9%）患者的 IDRF 减少，47 名（75.8%）肿瘤的 Nec >50%，27 名患者出现 31 种手术并发症。大多数 (83.9%) 在手术中继续患有 IDRF。常见的 IDRF 包绕肾蒂 (n=50)、肠系膜上动脉 (n=46)、腹腔干 (n=45) 和主动脉/腔静脉 (n=44)，并且大多数难以解决。TVR >65% 的患者与 Nec >50%（87.5% 对 54.5%，p=0.004）和 IDRF 减少（46.3% 对 19%，p=0.035）相关，但与手术并发症的发生率无关。结论 大多数 MYCNA 神经母细胞瘤具有高 TVR、减少 IDRF 和高 Nec 的高化学敏感性，从而为手术切除创造了有利条件。术前化疗通常难以治愈的不良反应者和持续性 IDRFs 仍然是外科手术的挑战。