Cheng Jian, Liu Wenke, Hui Xuhui, Zhang Si, Ju Yan
Department of Neurosurgery West China Hospital, Sichuan University, 37 Guo Xue Xiang, Wu Hou District, Chengdu, 610041, Sichuan, People's Republic of China.
Acta Neurochir (Wien). 2017 Sep;159(9):1603-1611. doi: 10.1007/s00701-017-3275-0. Epub 2017 Jul 27.
Pediatric hemangioblastomas are rare, and the clinical features, timing of surgical intervention, optimal treatment, and clinical outcomes are still unclear.
We performed a retrospective study of all patients with CNS hemangioblastomas who were treated at West China Hospital from January 2003 to March 2015. Patients under the age of 16 years were included in the study. The medical records of these patients were reviewed and statistically analyzed.
Twenty-five children (15 females and ten males, [mean age 12.6 ± 4.7 years, range 1-16 years]) presented with hemangioblastomas. Tumors were detected in the cerebellum, brainstem, and spinal cord in 40, 28, and 32% of patients, respectively. Sixteen children (64%) had VHL syndrome. The most frequent symptoms were those related to increased intracranial pressure. The mean duration of symptoms was 1.5 ± 2.1 months. Preoperative hydrocephalus was noted in 11 children (44%). Gross total resection was achieved in all children. Clinical symptoms improved in 19 children (76%), unchanged in four children (16%), and aggravated in two children (8%), respectively. The mean follow-up was 44.5 ± 32.3 months. Five patients (20%) experienced disease progression. Using univariate analysis, both tumor-associated cysts (P = 0.027) and VHL disease (P = 0.032) were significantly related to postoperative outcomes.
Pediatric hemangioblastomas have many different clinical features compared with adult cases. A high degree of suspicion for VHL disease should be raised in pediatric hemangioblastomas. Despite many challenges involved, surgical outcomes for pediatric hemangioblastomas are favorable. Lifelong follow-up is mandatory to detect the disease progression.
小儿血管母细胞瘤较为罕见，其临床特征、手术干预时机、最佳治疗方法及临床结局仍不明确。
我们对2003年1月至2015年3月在华西医院接受治疗的所有中枢神经系统血管母细胞瘤患者进行了一项回顾性研究。纳入研究的患者年龄在16岁以下。对这些患者的病历进行了回顾和统计分析。
25例儿童（15例女性，10例男性，平均年龄12.6±4.7岁，范围1 - 16岁）患有血管母细胞瘤。肿瘤分别在40%、28%和32%的患者中位于小脑、脑干和脊髓。16例儿童（64%）患有VHL综合征。最常见的症状是与颅内压升高相关的症状。症状的平均持续时间为1.5±2.1个月。11例儿童（44%）术前存在脑积水。所有儿童均实现了肿瘤全切。19例儿童（76%）临床症状改善，4例儿童（16%）症状无变化，2例儿童（8%）症状加重。平均随访时间为44.5±32.3个月。5例患者（20%）出现疾病进展。单因素分析显示，肿瘤相关囊肿（P = 0.027）和VHL病（P = 0.032）均与术后结局显著相关。
与成人病例相比，小儿血管母细胞瘤具有许多不同的临床特征。小儿血管母细胞瘤应高度怀疑VHL病。尽管存在诸多挑战，但小儿血管母细胞瘤的手术效果良好。必须进行终身随访以检测疾病进展。
