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Pediatric neuroblastoma with intraspinal extension: the role of surgical management
Journal of Neurosurgery: Pediatrics ( IF 1.9 ) Pub Date : 2023-12-15 , DOI: 10.3171/2023.10.peds23144
Diana Chang 1 , Taemin Oh 2 , Jarod L. Roland 3 , Katherine K. Matthay 4 , Kieuhoa T. Vo 4 , Caleb S. Edwards 2 , Peter P. Sun 2 , Kurtis I. Auguste 2 , Nalin Gupta 2
Affiliation  

OBJECTIVE

Neuroblastoma with spinal involvement accounts for up to 30% of pediatric spinal tumors and can cause profound neurological deficits. Chemotherapy is the preferred treatment option, but in select patients resection may be indicated. The goal of this study was to identify preoperative factors that led to early surgical intervention, with a specific emphasis on identifying differences on long-term neurological function and spinal deformity in the recent treatment era.

METHODS

A retrospective chart review was performed on all children diagnosed with neuroblastoma at a single institution from 2007 to 2020. Patient demographics, symptoms (motor deficit and sphincter dysfunction), and tumor characteristics (e.g., 123I metaiodobenzylguanidine [MIBG] avidity, MYCN amplification, chromosomal abnormality, pathology, catecholamine secretion, and stage) were recorded. Spine involvement included neural or vertebral extension, spinal cord compression, and/or T2 signal change on MRI. Survival, neurological status (motor deficit, sphincter dysfunction), and spine deformity at last follow-up were compared using univariate and multivariate analyses. The variables that contributed to neurological and deformity outcome were assessed with binomial logistic and linear regression models using R software.

RESULTS

Seventy-seven of the 160 patients with neuroblastoma had spinal neuroblastoma, meaning either bone metastases alone (n = 43) or intraspinal extension with or without neurological deficit (n= 34). Most patients with spinal neuroblastoma were treated with chemotherapy and/or radiation therapy (97% and 57%, respectively). Resection of the spinal tumor was performed in 14 (18%) patients, all of whom also received chemotherapy. Between the surgical and nonsurgical patients, no baseline demographic differences were found. However, surgical patients were more likely to present with either motor deficits (50% vs 5%, p = 0.0011) or bladder/bowel dysfunction (14% vs 0%, p 0.035), and a shorter median time to onset of neurological symptoms (33 vs 80 days, p = 0.0096). Surgical patients also had a significantly shorter median overall survival (33.0 vs 54 months, p = 0.014). Of the 14 patients who underwent spine surgery, 2 patients underwent surgery at the time of diagnosis while the remaining 12 underwent initial chemotherapy followed later by resection. The 2 patients who underwent initial surgery had excellent outcomes, with neither long-term motor or bowel/bladder deficits nor spinal deformity.

CONCLUSIONS

Surgical patients had shorter overall survival. However, the 2 patients with radiographic evidence of cord compression and acute neurological symptom onset who underwent initial, immediate surgery within 3 days of diagnosis had fewer long-term neurological deficits than surgical patients who underwent initial trials of chemotherapy. Thus, acute decompression may provide benefit in carefully selected patients with acute neurological deficits and cord compression on imaging.



中文翻译:

脊柱内扩展的小儿神经母细胞瘤:手术治疗的作用

客观的

累及脊柱的神经母细胞瘤占儿童脊柱肿瘤的 30%,可导致严重的神经功能缺损。化疗是首选治疗选择,但对于某些特定患者,可能需要进行切除术。本研究的目的是确定导致早期手术干预的术前因素,特别强调确定近期治疗时代长期神经功能和脊柱畸形的差异。

方法

对 2007 年至 2020 年在同一机构诊断为神经母细胞瘤的所有儿童进行了回顾性图表审查。患者人口统计、症状(运动缺陷和括约肌功能障碍)和肿瘤特征(例如123 I 间碘苄胍 ​​[MIBG] 亲合力、MYCN扩增、记录染色体异常、病理、儿茶酚胺分泌和分期)。脊柱受累包括神经或椎体伸展、脊髓受压和/或 MRI 上的 T2 信号变化。使用单变量和多变量分析比较末次随访时的生存率、神经系统状态(运动缺陷、括约肌功能障碍)和脊柱畸形。使用 R 软件通过二项式逻辑和线性回归模型评估影响神经和畸形结果的变量。

结果

160 名神经母细胞瘤患者中有 77 名患有脊髓神经母细胞瘤,这意味着要么是单纯的骨转移(n = 43),要么是伴有或不伴有神经功能缺损的脊柱内扩展(n = 34)。大多数脊髓神经母细胞瘤患者接受化疗和/或放射治疗(分别为 97% 和 57%)。14 名患者 (18%) 接受了脊柱肿瘤切除术,所有患者都接受了化疗。在手术患者和非手术患者之间,没有发现基线人口统计学差异。然而,手术患者更有可能出现运动缺陷(50% vs 5%,p = 0.0011)或膀胱/肠道功能障碍(14% vs 0%,p = 0.035),并且出现神经系统症状的中位时间较短(33 天与 80 天,p = 0.0096)。手术患者的中位总生存期也显着缩短(33.0 个月与 54 个月,p = 0.014)。在接受脊柱手术的 14 名患者中,2 名患者在诊断时接受了手术,其余 12 名患者接受了初步化疗,随后进行了切除。接受初次手术的 2 名患者预后良好,既没有长期运动或肠/膀胱缺陷,也没有脊柱畸形。

结论

手术患者的总生存期较短。然而,2 名放射学证据显示脊髓受压和急性神经系统症状发作的患者,在诊断后 3 天内接受了初次立即手术,其长期神经功能缺损的程度比接受初始化疗试验的手术患者要少。因此,急性减压可能为精心挑选的患有急性神经功能缺损和影像学脊髓受压的患者带来益处。

更新日期:2023-12-15
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