We reviewed our experience in managing of NF2-associated vestibular schwannoma (VS) in children and young adults regarding the effect of surgery and postoperative bevacizumab treatment. A total of 579 volumetric and hearing data sets were analyzed. The effect of surgery on tumor volume and growth rate was investigated in 46 tumors and on hearing function in 39 tumors. Long-term hearing follow-up behavior was compared with 20 non-operated ears in additional 15 patients. Sixteen operated VS were treated with bevacizumab. Mutation analysis of the NF2 gene was performed in 25 patients. Surgery significantly slowed down VS growth rate. Factors associated with a higher growth rate were increasing patient age, tumor volume, and constitutional truncating mutations. Immediately after surgery, functional hearing was maintained in 82% of ears. Deterioration of hearing was associated with initial hearing quality, larger tumor volumes, and larger resection amounts. Average hearing scores were initially better in the group of non-operated VS. Over time, hearing scores in both groups worsened with a similar dynamic. During bevacizumab treatment of residual tumors, four different patterns of growth were observed. Decompression of the internal auditory canal with various degrees of tumor resection decreases the postoperative tumor growth rates. Carefully tailored BAEP-guided surgery does not cause additional hearing deterioration. Secondary bevacizumab treatment showed heterogenous effects both regarding tumor size and hearing preservation. It seems that postoperative tumor residuals, that grow slower, behave differently to bevacizumab than reported for not-operated faster growing VS.

我们回顾了我们在治疗儿童和年轻成人 NF2 相关前庭神经鞘瘤 (VS) 方面关于手术和术后贝伐单抗治疗效果的经验。总共分析了 579 个体积和听力数据集。在 46 个肿瘤中研究了手术对肿瘤体积和生长速度的影响，在 39 个肿瘤中研究了对听力功能的影响。将另外 15 名患者的长期听力随访行为与 20 只未手术的耳朵进行了比较。用贝伐单抗治疗了 16 例手术 VS。对 25 名患者进行了 NF2 基因的突变分析。手术显着减缓了VS的增长速度。与较高生长率相关的因素是增加患者年龄、肿瘤体积和体质截断突变。术后即刻，82% 的耳朵保持功能性听力。听力下降与初始听力质量、较大的肿瘤体积和较大的切除量有关。非手术 VS 组的平均听力分数最初更好。随着时间的推移，两组的听力分数都以相似的动态恶化。在残留肿瘤的贝伐单抗治疗期间，观察到四种不同的生长模式。不同程度的肿瘤切除对内耳道进行减压会降低术后肿瘤的生长速度。精心定制的 BAEP 引导手术不会导致额外的听力下降。二次贝伐单抗治疗在肿瘤大小和听力保护方面均表现出异质性影响。似乎生长较慢的术后肿瘤残留物与贝伐单抗的表现不同，而不是报道的未手术生长较快的 VS。