Bleeker Gitta, van Eck-Smit Berthe L, Zwinderman Koos H, Versteeg Rogier, van Noesel Max M, Kam Boen L, Kaspers Gertjan J, van Schie Annelies, Kreissman Susan G, Yanik Gregory, Hero Barbara, Schmidt Matthias, Laureys Geneviève, Lambert Bieke, Øra Ingrid, Schulte Johannes H, Caron Huib N, Tytgat Godelieve A
Department of Paediatric Oncology, Academic Medical Centre/Emma Children's Hospital, PO Box 22700, 1100 DE, Amsterdam, The Netherlands.
Eur J Nucl Med Mol Imaging. 2015 Feb;42(2):222-30. doi: 10.1007/s00259-014-2909-1. Epub 2014 Sep 30.
The aim of this study was to find clinically relevant MIBG-avid metastatic patterns in patients with newly diagnosed stage 4 neuroblastoma.
Diagnostic (123)I-MIBG scans from 249 patients (123 from a European and 126 from the COG cohort) were assessed for metastatic spread in 14 body segments and the form of the lesions: "focal" (clear margins distinguishable from adjacent background) or "diffuse" (indistinct margins, dispersed throughout the body segment). The total numbers of diffuse and focal lesions were recorded. Patients were then categorized as having lesions exclusively focal, lesions more focal than diffuse, lesions more diffuse than focal, or lesions exclusively diffuse.
Diffuse lesions affected a median of seven body segments and focal lesions a median of two body segments (P < 0.001, both cohorts). Patients with a focal pattern had a median of 2 affected body segments and those with a diffuse pattern a median of 11 affected body segments (P < 0.001, both cohorts). Thus, two MIBG-avid metastatic patterns emerged: "limited-focal" and "extensive-diffuse". The median numbers of affected body segments in MYCN-amplified (MNA) tumours were 5 (European cohort) and 4 (COG cohort) compared to 9 and 11, respectively, in single-copy MYCN (MYCNsc) tumours (P < 0.001). Patients with exclusively focal metastases were more likely to have a MNA tumour (60% and 70%, respectively) than patients with the other types of metastases (23% and 28%, respectively; P < 0.001). In a multivariate Cox regression analysis, focal metastases were associated with a better event-free and overall survival than the other types of metastases in patients with MNA tumours in the COG cohort (P < 0.01).
Two metastatic patterns were found: a "limited and focal" pattern found mainly in patients with MNA neuroblastoma that correlated with prognosis, and an "extensive and diffuse" pattern found mainly in patients with MYCNsc neuroblastoma.
本研究旨在探寻新诊断的4期神经母细胞瘤患者中具有临床相关性的间碘苄胍（MIBG）摄取阳性转移模式。
对249例患者（欧洲队列123例，儿童肿瘤协作组[COG]队列126例）的诊断性（123）I-MIBG扫描进行评估，观察其在14个身体部位的转移扩散情况以及病变形态：“局灶性”（边界清晰，可与相邻背景区分）或“弥漫性”（边界不清，散布于整个身体部位）。记录弥漫性和局灶性病变的总数。然后将患者分为仅具有局灶性病变、局灶性病变多于弥漫性病变、弥漫性病变多于局灶性病变或仅具有弥漫性病变。
弥漫性病变累及的身体部位中位数为7个，局灶性病变为2个（两个队列P均<0.001）。具有局灶性模式的患者受累身体部位中位数为2个，具有弥漫性模式的患者为11个（两个队列P均<0.001）。由此出现了两种MIBG摄取阳性转移模式：“局限性局灶性”和“广泛性弥漫性”。与MYCN单拷贝（MYCNsc）肿瘤相比，MYCN扩增（MNA）肿瘤受累身体部位的中位数在欧洲队列中为5个，在COG队列中为4个，而在MYCNsc肿瘤中分别为9个和11个（P<0.001）。与其他类型转移的患者（分别为23%和28%）相比，仅具有局灶性转移的患者更有可能患有MNA肿瘤（分别为60%和70%；P<0.001）。在多因素Cox回归分析中，在COG队列的MNA肿瘤患者中，局灶性转移与更好的无事件生存期和总生存期相关（P<0.01）。
发现了两种转移模式：主要在与预后相关的MNA神经母细胞瘤患者中发现的“局限性和局灶性”模式，以及主要在MYCNsc神经母细胞瘤患者中发现的“广泛性和弥漫性”模式。
