Department of Radiology, the Third People's Hospital of Chengdu, 82 Qinglong Street, Chengdu, 610031, China.
Department of Radiology, West China Second University Hospital of Sichuan University, Chengdu, China.
Eur J Pediatr. 2023 May;182(5):2189-2196. doi: 10.1007/s00431-023-04899-0. Epub 2023 Mar 1.
Image-defined risk factors (IDRF) in neuroblastoma have been developed to predict tumor resectability and surgical complications; however, the potential prognostic value of IDRF in neuroblastoma has been variably reported. Previous studies did not report the IDRF status separately from the International Neuroblastoma Risk Group (INRG) stage. Moreover, the association between IDRF and clinical and pathological factors has not been discussed further. In this retrospective study, we investigated the clinical and biological features of neuroblastoma at different INRG stages based on IDRF. Event-free survival (EFS) and overall survival (OS) related to the INRG stage were analyzed using log-rank tests, and the prognostic value of the IDRF number and type was also evaluated. Among 72 patients, 182 IDRF at diagnosis were found in 79.2%. The distribution of the INRG stages was 10 L1 (13.9.0%), 25 L2 (34.7%), and 37 M/MS (51.4%). Patients with stage M/Ms had a larger tumor volume, a higher percentage of age ≥ 18 months, elevated lactate dehydrogenase (LDH) level, elevated ferritin level, and a higher percentage of COG high-risk compared with stage L1 and L2 patients. EFS and OS were similar for stage L1 and L2 tumors but were significantly poorer for metastatic disease. However, EFS (P = 0.06) and OS (P = 0.07) were similar for IDRF-negative and positive neuroblastomas. Patients with stage M/Ms with IDRF-positive had poorer EFS (P = 0.001) and OS (P < 0.001) compared with patients in stage L2. An IDRF ≥ 4, vascular IDRF, and infiltrative IDRF of the tumor were significant indicators of poor prognosis. Conclusion: Our study indicates that increasing the INRG stages based on IDRF is associated with various unfavorable clinical features of neuroblastoma. The principal determinant of survival in neuroblastoma is the presence of metastatic disease more than IDRF alone at diagnosis. Both the number and type of IDRF have important clinical significance in the protocol planning of neuroblastoma, rather than just considering the absence or presence of IDRF. What is Known: • The International Neuroblastoma Risk Group Staging System (INRGSS) now employs image-defined risk factors (IDRFs) to stratify and stage disease. • The presence of IDRF at diagnosis are associated with higher rates of operative complications and incomplete surgical resection. What is New: • The principal determinant of survival from neuroblastoma is the presence of metastatic disease at diagnosis, more than IDRF alone. • IDRF number and type should also be considered during the diagnosis and treatment planning of neuroblastoma, rather than just considering the absence or presence of IDRF.
图像定义的风险因素（IDRF）已被开发用于预测肿瘤的可切除性和手术并发症；然而，IDRF 在神经母细胞瘤中的潜在预后价值存在差异。以前的研究没有将 IDRF 状态与国际神经母细胞瘤风险组（INRG）分期分开报告。此外，IDRF 与临床和病理因素之间的关系尚未进一步讨论。在这项回顾性研究中，我们根据 IDRF 研究了不同 INRG 分期的神经母细胞瘤的临床和生物学特征。使用对数秩检验分析与 INRG 分期相关的无事件生存（EFS）和总生存（OS），并评估 IDRF 数量和类型的预后价值。在 72 名患者中，79.2%的患者在诊断时发现了 182 个 IDRF。INRG 分期的分布为 10 个 L1（13.9.0%）、25 个 L2（34.7%）和 37 个 M/MS（51.4%）。与 L1 和 L2 期患者相比，M/MS 期患者的肿瘤体积更大，年龄≥18 个月的比例更高，乳酸脱氢酶（LDH）水平升高，铁蛋白水平升高，COG 高危比例更高。L1 和 L2 期肿瘤的 EFS 和 OS 相似，但转移性疾病的 EFS 和 OS 明显较差。然而，IDRF 阴性和阳性神经母细胞瘤的 EFS（P=0.06）和 OS（P=0.07）相似。M/MS 期 IDRF 阳性患者的 EFS（P=0.001）和 OS（P<0.001）均较 L2 期患者差。IDRF≥4、血管 IDRF 和肿瘤浸润性 IDRF 是预后不良的显著指标。结论：本研究表明，基于 IDRF 增加 INRG 分期与神经母细胞瘤各种不良临床特征相关。神经母细胞瘤生存的主要决定因素是存在转移性疾病，而不仅仅是 IDRF 本身。IDRF 的数量和类型在神经母细胞瘤的方案规划中具有重要的临床意义，而不仅仅是考虑 IDRF 的存在或不存在。已知的：•国际神经母细胞瘤风险组分期系统（INRGSS）现在采用图像定义的风险因素（IDRF）对疾病进行分层和分期。•诊断时存在 IDRF 与更高的手术并发症发生率和不完全手术切除率相关。新的：•神经母细胞瘤生存的主要决定因素是诊断时存在转移性疾病，而不仅仅是 IDRF 本身。•在神经母细胞瘤的诊断和治疗计划中，还应考虑 IDRF 的数量和类型，而不仅仅是考虑 IDRF 的存在或不存在。
