Zipfel Julian, Gorbachuk Mykola, Gugel Isabel, Tatagiba Marcos, Schuhmann Martin U
Section of Pediatric Neurosurgery, Department of Neurosurgery, University Hospital Tübingen, 72076 Tübingen, Germany.
Children (Basel). 2022 Apr 1;9(4):490. doi: 10.3390/children9040490.
Vestibular schwannomas (VS) usually manifest between the 5th and 8th decade of life. Most pediatric cases are associated with Neurofibromatosis type 2 and sporadic VS are rare in this age group. Few case series have been published. We report on our institutional series of sporadic VS in children. We included all cases between 2003 and 2021; 28 of 1635 patients harbored a sporadic VS and were younger than 21 years old. A retrospective review of clinical parameters and surgical data as well as outcomes was performed. All procedures were performed via a retrosigmoid approach. Preoperative imaging was assessed, and tumor volumetry was performed. Mean follow-up was 28 months, symptomatology was diverse. Most children and adolescents presented with hearing loss and tinnitus. All cases with multiple preoperative magnetic resonance imaging scans showed volumetric tumor growth between 1 and 18%/month (mean 8.9 ± 5.6%). Cystic tumor morphology and bone erosion was seen in larger tumors. Gross total resection was possible in 78% of patients and no recurrence was observed. All patients with subtotal resection showed tumor regrowth. Sporadic VS in children are rare and present with a high clinical variability. Surgical resection is the primary therapy and is feasible with favorable results comparable to the adult age group.
前庭神经鞘瘤(VS)通常在50至80岁之间出现。大多数儿科病例与2型神经纤维瘤病相关,散发性VS在该年龄组中很少见。很少有病例系列发表。我们报告了我们机构的儿童散发性VS病例系列。我们纳入了2003年至2021年期间的所有病例;1635例患者中有28例患有散发性VS且年龄小于21岁。对临床参数、手术数据以及结果进行了回顾性分析。所有手术均通过乙状窦后入路进行。评估了术前影像学检查并进行了肿瘤体积测量。平均随访28个月,症状多样。大多数儿童和青少年表现为听力损失和耳鸣。所有术前进行多次磁共振成像扫描的病例显示肿瘤体积每月增长1%至18%(平均8.9±5.6%)。在较大的肿瘤中可见囊性肿瘤形态和骨质侵蚀。78%的患者实现了肿瘤全切,未观察到复发。所有次全切患者均出现肿瘤复发。儿童散发性VS很少见,临床表现高度可变。手术切除是主要治疗方法,可行且效果良好,与成人年龄组相当。