影像定义的风险因素与神经母细胞瘤的临床表现,肿瘤生物学和预后的关联:单中心回顾性研究。
Association of image-defined risk factors with clinical features, tumor biology, and outcomes in neuroblastoma: a single-center retrospective study.
发表日期:2023 Mar 01
作者:
Dan-Dan Yang, Chuan Liu, Jin Gao, Qiao-Jun Hu, Yong Liang, Jian Liu
来源:
EUROPEAN JOURNAL OF PEDIATRICS
摘要:
图像定义的风险因素(IDRF)已被开发用于预测神经母细胞瘤的肿瘤可切性和手术并发症; 然而,神经母细胞瘤中IDRF的潜在预后价值已有不同程度的报道。以往的研究没有单独报告IDRF的状况,而只是将其纳入国际神经母细胞瘤风险组(INRG)阶段的范畴中。此外,IDRF与临床和病理因素之间的关系也没有进一步讨论。在这项回顾性研究中,我们根据IDRF调查了不同INRG阶段神经母细胞瘤的临床和生物学特征。使用log-rank检验分析与INRG阶段相关的无事件生存期(EFS)和总生存期(OS),同时也评估了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阶段的分布是10例L1(13.9.0%)、25例L2(34.7%)、37例M/MS(51.4%)。与L1和L2患者相比,M/Ms阶段的患者具有更大的肿瘤体积、年龄≥18个月的比例更高、乳酸脱氢酶(LDH)水平升高、铁蛋白水平升高以及COG高风险百分比更高。对于L1和L2肿瘤,EFS和OS相似,而转移病情的结果则显著较差。然而,IDRF阴性和阳性神经母细胞瘤的EFS(P=0.06)和OS(P=0.07)却相似。在IDRF阳性的M/Ms阶段患者中,其EFS(P=0.001)和OS(P<0.001)则相对较差。瘤中IDRF≥4、血管IDRF和浸润性IDRF是神经母细胞瘤不良预后的重要指标。总之,我们的研究表明,基于IDRF增加INRG阶段与神经母细胞瘤的各种不良临床特征有关。神经母细胞瘤生存的主要决定因素是转移病情而不是单纯的IDRF诊断。在神经母细胞瘤的诊断和治疗规划中,IDRF数量和类型都应被考虑在内,而不仅仅是考虑其有无。已知:国际神经母细胞瘤风险分组分期系统(INRGSS)现在采用图像定义的风险因素(IDRF)来分层和分期疾病。IDRF诊断时的存在与手术并发症和不完全切除的风险较高有关。新发现:神经母细胞瘤生存的主要决定因素是诊断时的转移病情,而不是单纯的IDRF。在神经母细胞瘤的诊断和治疗规划中,应考虑IDRF数量和类型,而不仅仅是它们的有无。©2023年作者,独家许可Springer-Verlag GmbH Germany,属Springer Nature。
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.© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.