Peuchmaur Michel, d'Amore Emanuele S G, Joshi Vijay V, Hata Jun-ichi, Roald Borghild, Dehner Louis P, Gerbing Robert B, Stram Daniel O, Lukens John N, Matthay Katherine K, Shimada Hiroyuki
Service de Pathologie, Hôpital Robert Debré, Paris, France.
Cancer. 2003 Nov 15;98(10):2274-81. doi: 10.1002/cncr.11773.
Ganglioneuroblastoma, nodular (GNBn) comprises one of the categories of peripheral neuroblastic tumors. All tumors in this category, according to the original International Neuroblastoma Pathology Classification, are classified into an unfavorable histology group. Subsequently, it has been reported that GNBn can be divided into two prognostic subsets, a favorable subset (FS) and an unfavorable subset (US).
Histology slides from 70 patients who were enrolled in Children's Cancer Group studies 3881 and 3891 and who had a diagnosis of GNBn were reviewed jointly by the members of International Neuroblastoma Pathology Committee (INPC): 1) to confirm the diagnosis of GNBn, 2) to identify the FS and US by applying the same age-linked criteria that were used to distinguish the favorable histology group and unfavorable histology group in conventional neuroblastoma tumors from the neuroblastomatous component of GNBn tumors, and 3) to verify the significant prognostic difference between these two subsets. The patients had been used in a previous study, and survival data for the patients were updated since the time of their last report.
The review clarified and illustrated morphologic characteristics of classical GNBn and it variants. The diagnosis of GNBn was confirmed in 67 of 70 patients. There were 22 patients with GNBn in the FS and 45 patients with GNBn in the US. The estimated survival differences between the FS and US patients with GNBn were statistically significant (8-year event free survival rate: 86.1% vs. 32.2%; P = 0.0003; overall survival rate: 90.5% vs. 33.2%; P = 0.0003).
This study confirmed the recently defined prognostic subsets of GNBn. The INPC proposes to modify the International Neuroblastoma Pathology Classification by distinguishing the FS and the US among patients with GNBn tumors.
结节性神经节神经母细胞瘤（GNBn）是周围神经母细胞瘤的一种类型。根据最初的国际神经母细胞瘤病理分类，该类别中的所有肿瘤均被归类为预后不良的组织学类型。随后，有报道称GNBn可分为两个预后亚组，即预后良好亚组（FS）和预后不良亚组（US）。
国际神经母细胞瘤病理委员会（INPC）的成员联合审查了70例参加儿童癌症组研究3881和3891且诊断为GNBn的患者的组织学切片：1）确认GNBn的诊断；2）通过应用与区分传统神经母细胞瘤肿瘤中预后良好组织学类型和预后不良组织学类型相同的年龄相关标准，从GNBn肿瘤的神经母细胞瘤成分中识别出FS和US；3）验证这两个亚组之间的显著预后差异。这些患者曾用于先前的一项研究，自上次报告以来对患者的生存数据进行了更新。
审查明确并阐述了经典GNBn及其变体的形态学特征。70例患者中有67例确诊为GNBn。FS中有22例GNBn患者，US中有45例GNBn患者。GNBn的FS和US患者之间的估计生存差异具有统计学意义（8年无事件生存率：86.1%对32.2%；P = 0.0003；总生存率：90.5%对33.2%；P = 0.0003）。
本研究证实了最近定义的GNBn预后亚组。INPC建议修改国际神经母细胞瘤病理分类，在GNBn肿瘤患者中区分FS和US。
