Outcomes of patients with intermediate-risk neuroblastoma presenting with motor deficits relating to intraspinal tumor extension: A report from the Children's Oncology Group study ANBL0531

IF 2.4 3区医学 Q2 HEMATOLOGY

Pediatric Blood & Cancer Pub Date : 2024-11-06 DOI:10.1002/pbc.31407

Julie Voeller, Howard M. Katzenstein, Arlene Naranjo, Sheena C. Tenney, Lulu Chen, Wendy B. London, Michael H. Handler, Mary Lou Schmidt, Hiroyuki Shimada, Michael D. Hogarty, Julie Gastier-Foster, Julie R. Park, Susan L. Cohn, John M. Maris, Rochelle Bagatell, Clare J. Twist

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Abstract

Background

Tumor invasion of the spinal canal is detected radiographically in approximately 15% of patients with newly diagnosed neuroblastoma (NB). The optimal clinical approach to maintain excellent survival outcomes while minimizing long-term sequelae is yet to be defined.

Methods

Patients with intermediate-risk neuroblastoma (IR-NB) and radiographically identified intraspinal tumors who were treated on the Children's Oncology Group study ANBL0531 were studied prospectively to evaluate neurologic outcomes related to cord compression. Patients were defined as being symptomatic versus asymptomatic based on reporting of neurologic motor deficits at diagnosis. Patient characteristics, tumor biology, chemotherapy treatment, surgical interventions, and neurologic and disease outcomes are reported.

Results

Of the 92 patients with intraspinal tumors, 42 (46%) were symptomatic and most (73%) had complete resolution of symptoms. Age, degree of motor deficit, and duration of symptoms at diagnosis were not associated with complete resolution. While symptomatic patients were more likely to undergo upfront laminectomy, laminectomy was not associated with improvement of motor symptoms. Administration of additional chemotherapy beyond initial treatment assigned per protocol to achieve the treatment end point was not associated with achieving symptom resolution.

Conclusion

Patients presenting with motor deficits due to intraspinal tumor had excellent survival and favorable neurologic outcomes, with the majority reporting complete resolution of motor symptoms regardless of severity and duration of symptoms at diagnosis or neurosurgical intervention. Prompt diagnosis and initiation of first-line chemotherapy treatment remain priority, while neurosurgical intervention should be reserved for patients with rapid neurologic deterioration. Biology-based therapy and tumor response should continue to be used to maintain favorable outcomes.

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因脊髓内肿瘤扩展而出现运动障碍的中危神经母细胞瘤患者的预后：儿童肿瘤集团 ANBL0531 研究报告。

背景：在新确诊的神经母细胞瘤（NB）患者中，约15%的患者可通过影像学检查发现肿瘤侵犯椎管。既要保持良好的存活率，又要尽量减少长期后遗症的最佳临床方法尚未确定：方法：我们对中危神经母细胞瘤（IR-NB）患者和经放射学检查发现的椎管内肿瘤患者进行了前瞻性研究，以评估与脊髓压迫相关的神经系统预后。根据诊断时神经系统运动障碍的报告，将患者定义为有症状和无症状患者。报告了患者特征、肿瘤生物学特性、化疗治疗、手术干预以及神经和疾病预后：在92例椎管内肿瘤患者中，42例（46%）无症状，大多数（73%）症状完全消失。诊断时的年龄、运动障碍程度和症状持续时间与症状完全缓解无关。虽然有症状的患者更有可能接受前期椎板切除术，但椎板切除术与运动症状的改善无关。为达到治疗终点而在初始治疗方案之外追加化疗与症状缓解无关：结论：因椎管内肿瘤导致运动障碍的患者生存率极高，神经系统预后良好，无论诊断时症状的严重程度和持续时间或神经外科干预情况如何，大多数患者的运动症状都能完全缓解。及时诊断和启动一线化疗仍是当务之急，而神经外科干预应保留给神经功能急剧恶化的患者。应继续采用基于生物学的疗法和肿瘤反应来保持良好的预后。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Blood & Cancer 医学-小儿科

CiteScore

4.90

自引率

9.40%

发文量

546

审稿时长

1.5 months

期刊介绍： Pediatric Blood & Cancer publishes the highest quality manuscripts describing basic and clinical investigations of blood disorders and malignant diseases of childhood including diagnosis, treatment, epidemiology, etiology, biology, and molecular and clinical genetics of these diseases as they affect children, adolescents, and young adults. Pediatric Blood & Cancer will also include studies on such treatment options as hematopoietic stem cell transplantation, immunology, and gene therapy.