Surgical management and outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed
Surgical management and outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed
复制标题
神经节神经瘤和混合神经母细胞瘤的手术治疗和结果。
DOI：
10.1007/s00383-017-4100-9
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发表时间：
2017-09-01
影响因子：
1.8
通讯作者：
Zou, Yan
中科院分区：
文献类型：
--
作者：
Yang, Tianyou;Huang, Yongbo;Zou, Yan
关于节细胞神经瘤和混合型节细胞神经母细胞瘤的治疗和预后的临床研究有限。我们报告了一家机构中神经节细胞神经瘤和神经节细胞神经母细胞瘤混合型的手术结果。回顾性分析了2009年5月至2015年5月在三级儿童医院诊断和切除的神经节细胞神经瘤和神经节细胞神经母细胞瘤混合型。收集患者的人口统计学资料、INSS分期、手术并发症、残留肿瘤大小和预后。所有患者均患有局部肿瘤，并接受了手术治疗。总体急性并发症发生率为8.8%（3/34），无致死性并发症。34例患者中有33例至少进行了肉眼肿瘤切除。术后X线检查发现肿瘤残留者6例，无残留者25例，无残留者3例。33例（97.1%）患者在中位随访36个月（范围1-82）期间存活。在亚组分析中，神经节细胞瘤和神经节细胞母细胞瘤混合型之间在手术并发症和生存率方面没有显著差异。与腹部肿瘤相比，胸部肿瘤的完全切除率增加（p = 0.03）。然而，在有残留肿瘤的患者和没有残留肿瘤的患者之间，总生存率没有显著差异（p = 0.089）。6例残留肿瘤患者中，3例完全消退，2例无变化，1例于首次手术后3年死亡（本研究中唯一死亡），节细胞神经瘤和节细胞神经母细胞瘤混合瘤可安全有效地切除，残留肿瘤似乎不影响总生存率。
Clinical researches about the management and outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed are limited. We report the surgical outcomes of ganglioneuroma and ganglioneuroblastoma-intermixed in a single institution.Ganglioneuroma and ganglioneuroblastoma-intermixed diagnosed and resected between May 2009 and May 2015 in a tertiary children's hospital were retrospectively reviewed. Patients' demographic data, INSS stage, surgical complications, residual tumor size and outcomes were collected.Thirty-four patients were included in the current study. All had localized tumors and were surgically managed. The overall acute complications rates were 8.8% (3/34) and none were fatal. Thirty-three of 34 patients had at least macroscopic tumor resection. Six patients had radiographically detected residual tumor after surgery, 25 none and 3 undocumented. Thirty-three (97.1%) patients were alive during a median follow-up of 36 months (range 1-82). In subgroup analysis, no significant difference regarding surgical complications and survival was found between ganglioneuroma and ganglioneuroblastoma-intermixed. Increased complete resection rates were observed in thoracic tumor compared with abdominal ones (p = 0.03). However, no significant difference (p = 0.089) regarding overall survival was found between patients with residual tumors and those without. Of the six patients with residual tumors, three showed complete resolution, two were unchanged and one died 3 years after initial surgery (the only death in this study).Ganglioneuroma and ganglioneuroblastoma-intermixed can be safely and effectively resected, the residual tumor seems not to influence overall survival.