Pediatric Surgery Unit, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, 16147, Genoa, Italy.
Oncology Unit, IRCCS Istituto Giannina Gaslini, Largo Gaslini 5, 16147, Genoa, Italy.
Pediatr Surg Int. 2021 Jan;37(1):37-47. doi: 10.1007/s00383-020-04766-1. Epub 2020 Oct 29.
To clarify the role of primary tumor resection in stage 4S neuroblastoma.
We investigated a cohort of 172 infants diagnosed with stage 4S neuroblastoma between 1994 and 2013. Of 160 evaluable patients, 62 underwent upfront resection of the primary tumor and 98 did not.
Five-year progression-free and overall survival were significantly better in those who had undergone upfront surgery (83.6% vs 64.2% and 96.8% vs 85.7%, respectively). One post-operative death and four non-fatal complications occurred in the resection group. Three patients who had not undergone resection died of chemotherapy-related toxicity. Thirteen patients underwent late surgery to remove a residual tumor, without complications: all but one alive. Outcomes were better in patients diagnosed from 2000 onwards.
Infants diagnosed with stage 4S neuroblastoma who underwent upfront tumor resection had a better outcome. However, this result cannot be definitely attributed to surgery, since these patients were selected on the basis of their favorable presenting features. Although the question of whether to operate or not at disease onset is still unsolved, this study confirms the importance of obtaining enough adequate tumor tissue to enable histological and biological studies to properly address treatment, to achieve the best possible outcome.
阐明 4S 期神经母细胞瘤原发肿瘤切除术的作用。
我们研究了 1994 年至 2013 年间诊断为 4S 期神经母细胞瘤的 172 名婴儿。在 160 名可评估的患者中，62 名患者接受了原发肿瘤的 upfront 切除术，98 名患者未接受。
upfront 手术组的 5 年无进展生存率和总生存率明显更好（分别为 83.6%、64.2%和 96.8%、85.7%）。切除术组发生 1 例术后死亡和 4 例非致命性并发症。未接受切除术的 3 例患者因化疗相关毒性而死亡。13 例患者行晚期手术切除残余肿瘤，无并发症：除 1 例外均存活。2000 年以后诊断的患者结局更好。
接受 upfront 肿瘤切除术的 4S 期神经母细胞瘤婴儿的结局更好。然而，由于这些患者基于其有利的表现特征被选择，因此不能确定该结果归因于手术。尽管是否在疾病发作时进行手术的问题仍未解决，但本研究证实了获得足够充分的肿瘤组织以进行组织学和生物学研究的重要性，以实现最佳的治疗效果。
