儿童和青少年NF2相关前庭裂神经瘤的治疗:手术和临床因素对肿瘤体积和生长速度的影响。

Isabel Gugel, Florian Grimm, Christian Teuber, Lan Kluwe, Victor-Felix Mautner, Marcos Tatagiba, Martin Ulrich Schuhmann
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引用次数: 0

摘要

研究目的作者的目的是评估神经纤维瘤病2型(NF2)相关前庭分裂瘤(VSs)的肿瘤体积和生长速度,以及诊断时年龄小于25岁的儿童和成人手术前后的临床因素或突变类型:使用薄切片(< 3 mm)T1加权对比增强磁共振成像对28名NF2患者的46个手术肿瘤进行了579次体积测量。随访时间为 21 至 167 个月(平均 75 个月)。生长率通过多线性回归模型计算得出。对25名患者进行了NF2基因突变分析:结果:手术明显(p = 0.013)降低了 VS 的生长速度,从 0.69 ± 1.30 cm3/年降至 0.23 ± 0.42 cm3/年。与VS生长率较高明显相关的因素包括患者年龄增加(p < 0.0005)、肿瘤体积增大(p = 0.006)、肿瘤大小增大(p = 0.001)以及NF2基因中的宪法截断突变(p = 0.018)。脊髓上皮瘤患者和右侧肿瘤患者的VS生长率往往较高,而外周裂隙瘤患者的VS生长率较低;但没有统计学意义:有加速生长潜在危险因素(如体积大、截断突变)和年龄增大的患者在手术前后应更密切地监测。
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Management of NF2-associated vestibular schwannomas in children and young adults: influence of surgery and clinical factors on tumor volume and growth rate.

Objective: The authors' aim was to evaluate the tumor volume and growth rate of neurofibromatosis type 2 (NF2)-associated vestibular schwannomas (VSs) and the clinical factors or type of mutations before and after surgery in children and adults younger than 25 years at the time of diagnosis.

Methods: A total of 579 volumetric measurements were performed in 46 operated tumors in 28 NF2 patients, using thin-slice (< 3 mm) T1-weighted contrast-enhanced MRI. The follow-up period ranged from 21 to 167 months (mean 75 months). Growth rate was calculated using a multilinear regression model. Mutation analysis of the NF2 gene was performed in 25 patients.

Results: Surgery significantly (p = 0.013) slowed the VS growth rate from 0.69 ± 1.30 cm3/yr to 0.23 ± 0.42 cm3/yr. Factors significantly associated with a higher growth rate of VSs were increasing patient age (p < 0.0005), tumor volume (p = 0.006), tumor size (p = 0.001), and constitutional truncating mutations in the NF2 gene (p = 0.018). VS growth rates tended to be higher in patients with spinal ependymomas and in right-sided tumors and lower in the presence of peripheral schwannomas; however, no statistical significance was achieved.

Conclusions: Decompression of the internal auditory canal with various degrees of tumor resection decreases the postoperative tumor growth rate in children and young adults with NF2-associated VS. Patients with potential risk factors for accelerated growth (e.g., large volume, truncating mutations) and with increasing age should be monitored more closely before and after surgery.

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