Introduction

Choroid plexus tumors are uncommon intraventricular tumors that develop from the choroid plexus of the central nervous system. Choroid plexus papillomas arising from the cerebellopontine angle have been reported to almost exclusively occur in adults and are rarely found in children.

Methods

We report a systematic review conducted in accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines of SCOPUS and PubMed databases for case reports and case series of choroid plexus papillomas arising in the cerebellopontine angle in the pediatric population and discuss clinical presentation, imaging features, management options, and outcomes. We also report a case managed at our center.

Results

Ten cases of pediatric choroid plexus papillomas arising in the cerebellopontine angle were identified from the systematic review in addition to the case reported here, resulting in a total of eleven cases. The patients’ median age was 8 years with a slight female sex predilection (1.2:1). Patients most commonly presented with headache, cerebellar signs, and cranial nerve palsies with median duration of symptoms at 4 months. All patients underwent surgical treatment with majority achieving gross total excision. No deaths were reported at median follow-up of 12 months. Complete neurologic recovery was attained in seven cases while partial recovery was seen in two cases.

Conclusion

Choroid plexus papillomas found in the cerebellopontine angle in the pediatric population are extremely rare but they should be considered in the differential diagnosis. Complete surgical resection is the mainstay of treatment with excellent outcomes achievable in majority of patients.

中文翻译：

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小脑桥脑角引起的小儿脉络丛神经乳头状瘤：系统性回顾，并有病例说明。

介绍

脉络丛肿瘤是从中枢神经系统的脉络丛发展而来的不常见的脑室内肿瘤。据报道，小脑桥脑角引起的脉络丛乳头状瘤几乎只在成年人中发生，在儿童中很少见。

方法

我们报告根据SCOPUS和PubMed数据库的PRISMA（系统评价和荟萃分析的首选报告项目）指南进行的系统评价，以了解小儿桥小脑角中脉络丛乳头状瘤的病例报告和病例系列，并进行讨论临床表现，影像学特征，治疗选择和结果。我们还报告了在我们中心管理的案件。

结果

除本文报道的病例外，通过系统评价还鉴定出十例小脑桥角引起的小儿脉络丛神经乳头状瘤，共十一例。患者的中位年龄为8岁，女性偏爱轻微（1.2：1）。患者最常出现头痛，小脑体征和颅神经麻痹，其症状持续时间为4个月。所有患者均接受了外科手术治疗，其中大多数患者实现了总切除。中位随访12个月无死亡报告。7例达到了完全的神经恢复，而2例达到了部分恢复。

结论

小儿桥小脑角发现脉络膜乳头状瘤非常少见，但在鉴别诊断中应予以考虑。完整的手术切除是治疗的主要手段，大多数患者均可获得出色的治疗效果。