Department of Paediatric Oncology, Emma Children's Hospital, Academic Medical Centre (AMC), PO Box 22700, 1100 DE, Amsterdam, The Netherlands.
Eur J Nucl Med Mol Imaging. 2013 Oct;40(10):1516-22. doi: 10.1007/s00259-013-2455-2. Epub 2013 Jun 6.
In patients with localised neuroblastoma without adverse genetic aberrations, observational treatment is justified. Therapy is required when organ or respiratory functions have become compromised. As the outcome is good, side effects of treatment should be prevented. The aim of this retrospective study was to evaluate response and outcome in patients treated with (131)I-metaiodobenzylguanidine (MIBG) for unresectable localised neuroblastoma, with compromised organ functions.
Patients with localised neuroblastoma [median age 1.6 years (0-5.5 years)] diagnosed between 1989 and 2008 were included in this retrospective study (n = 21). Primary tumours were unresectable and there was a compromised organ or respiratory function. Diagnosis and staging were performed according to the International Neuroblastoma Staging System. Fixed doses of (131)I-MIBG therapy (50-200 mCi) were given. The median number of infusions was two (range one to seven). Response was graded according to the International Neuroblastoma Response Criteria.
Of the 21 patients, 14 did not need any chemotherapy. Patients were treated with (131)I-MIBG therapy and, in most cases, with additional surgery and/or chemotherapy. Sixteen achieved complete response (CR), three very good partial response (VGPR), one partial response (PR) and one progressive disease (PD). Two patients died of PD after having achieved CR initially and due to surgical complications a few months after resection. Ten-year overall survival and event-free survival were 90.5 %. The median follow-up was 8.5 years (range 0.4-19.6 years).
(131)I-MIBG therapy is an effective treatment modality for unresectable localised neuroblastoma with compromised organ functions. However, this was a small and heterogeneous cohort and further studies are needed.
对于无不良遗传异常的局限性神经母细胞瘤患者,观察治疗是合理的。当器官或呼吸功能受损时,需要进行治疗。由于预后良好,应预防治疗的副作用。本回顾性研究的目的是评估(131)I-间碘苄胍(MIBG)治疗功能受损的不可切除局限性神经母细胞瘤患者的反应和结果。
本回顾性研究纳入了 1989 年至 2008 年间诊断为局限性神经母细胞瘤的患者(n = 21),这些患者的年龄中位数为 1.6 岁(0-5.5 岁)。原发病灶不可切除,存在器官或呼吸功能受损。诊断和分期均按照国际神经母细胞瘤分期系统进行。给予固定剂量的(131)I-MIBG 治疗(50-200 mCi)。输注中位数为两次(范围一次至七次)。根据国际神经母细胞瘤反应标准对反应进行分级。
21 例患者中,有 14 例无需任何化疗。患者接受(131)I-MIBG 治疗,大多数患者还接受了额外的手术和/或化疗。16 例达到完全缓解(CR),3 例非常好的部分缓解(VGPR),1 例部分缓解(PR)和 1 例进展性疾病(PD)。2 例患者最初获得 CR 后死于 PD,且因手术后并发症,在切除后几个月死亡。10 年总生存率和无事件生存率为 90.5%。中位随访时间为 8.5 年(范围 0.4-19.6 年)。
(131)I-MIBG 治疗是治疗功能受损的不可切除局限性神经母细胞瘤的有效方法。然而,这是一个小型且异质性的队列,需要进一步研究。