Monclair Tom, Mosseri Véronique, Cecchetto Giovanni, De Bernardi Bruno, Michon Jean, Holmes Keith
Department of Hepatic, Gastrointestinal and Paediatric surgery, Oslo University Hospital - Rikshospitalet, Oslo, Norway.
Service of Statistics, Institut Curie, Paris, France.
Pediatr Blood Cancer. 2015 Sep;62(9):1536-42. doi: 10.1002/pbc.25460. Epub 2015 Feb 8.
The European multicenter study LNESG1 was designed to evaluate the safety and efficacy of surgical treatment alone in patients with localised neuroblastoma. In a retrospective, observational study we examined the impact of image-defined risk factors (IDRF) on operative complications and survival (EFS and OS).
534 patients with localised, non-MYCN amplified neuroblastoma were recruited between 1995 and 1999. Group 1 consisted of 291 patients without IDRF (Stage L1 in the International Neuroblastoma Risk Group (INRG) staging system), all treated with primary surgery. Group 2: 118 patients with IDRF (INRG Stage L2), also treated with primary surgery. Group 3: 125 patients in whom primary surgery was not attempted, 106 receiving neo-adjuvant chemotherapy.
In L1 patients (Group 1) 5-year EFS was 92% and OS 98%. In L2 patients (Group 2 and 3) EFS was 79% and OS 89%. The differences in both EFS and OS were significant. EFS and OS in Group 2 (86% and 95%) were significantly better than 73% and 83% in Group 3. In INSS stage 1, 2 and 3, EFS were respectively 94%, 81% and 76%. Except between stage 2 and 3 the differences were significant. OS were respectively 99%, 93% and 83%, all significantly different. The 17% operative complication rate in L2 patients was significantly higher than 5% in L1 patients.
In localised neuroblastoma, IDRF at diagnosis are associated with worse survival rates and higher rates of operative complications. The impact of IDRF should become an integrated part of therapy planning.
欧洲多中心研究 LNESG1 旨在评估单纯手术治疗局限性神经母细胞瘤患者的安全性和有效性。在一项回顾性观察研究中，我们研究了影像定义的风险因素（IDRF）对手术并发症和生存（无事件生存期和总生存期）的影响。
1995 年至 1999 年间招募了 534 例局限性、非 MYCN 扩增的神经母细胞瘤患者。第 1 组由 291 例无 IDRF 的患者组成（国际神经母细胞瘤风险组（INRG）分期系统中的 L1 期），均接受一期手术治疗。第 2 组：118 例有 IDRF 的患者（INRG L2 期），也接受一期手术治疗。第 3 组：125 例未尝试一期手术的患者，106 例接受新辅助化疗。
L1 期患者（第 1 组）5 年无事件生存期为 92%，总生存期为 98%。L2 期患者（第 2 组和第 3 组）无事件生存期为 79%，总生存期为 89%。无事件生存期和总生存期方面的差异均具有统计学意义。第 2 组的无事件生存期和总生存期（86%和 95%）显著优于第 3 组的 73%和 83%。在国际神经母细胞瘤分期系统（INSS）1 期、2 期和 3 期，无事件生存期分别为 94%、81%和 76%。除 2 期和 3 期之间外，差异均具有统计学意义。总生存期分别为 99%、93%和 83%，均有显著差异。L2 期患者 17%的手术并发症发生率显著高于 L1 期患者的 5%。
在局限性神经母细胞瘤中，诊断时的 IDRF 与较差的生存率和较高的手术并发症发生率相关。IDRF 的影响应成为治疗计划的一个组成部分。
