Effects of preoperative chemotherapy on neuroblastoma with MYCN amplification: a surgeon's perspective

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.

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.

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.

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.