关键词: Aicardi syndrome atypical choroid plexus infant oncology papilloma

来  源:   DOI:10.3171/2018.12.PEDS18453   PDF(Sci-hub)

Abstract:
Choroid plexus papillomas (CPPs) are rare, benign tumors that can arise in young children. Most pediatric patients present with signs of hydrocephalus and require immediate treatment. The natural history of choroid plexus tumors in children without hydrocephalus is poorly defined. In this report, the authors present the very rare case of a child without hydrocephalus but with two intraventricular choroid plexus tumors discovered shortly after birth. Initial imaging had been performed for seizures and showed agenesis of the corpus callosum and enhancing tumors in the third and left lateral ventricles. Sequential imaging demonstrated rapid growth of both tumors. The lateral tumor was removed when the child was 3 months of age. A histological examination of the specimen showed benign features with an elevated mitotic rate. Given the patient\'s age of under 3 years, the diagnosis was WHO grade I CPP. The third ventricle tumor grew rapidly. A second surgery was performed and this tumor was resected. Again, the pathological diagnosis was WHO grade I CPP. The authors present this rare case and discuss the current relevant literature.
摘要:
脉络丛乳头状瘤(CPPs)很少见,可以出现在幼儿中的良性肿瘤。大多数儿科患者出现脑积水的迹象,需要立即治疗。没有脑积水的儿童脉络丛肿瘤的自然史定义不清。在这份报告中,作者介绍了一个非常罕见的病例,一个没有脑积水的儿童,但在出生后不久发现了两个脑室内脉络丛肿瘤。已对癫痫发作进行了初步成像,并显示出call体发育不全并增强了第三和左侧脑室的肿瘤。序贯成像显示两种肿瘤的快速生长。当孩子3个月大时,切除了外侧肿瘤。标本的组织学检查显示良性特征,有丝分裂率升高。鉴于患者的年龄在3岁以下,诊断为WHOI级CPP.第三脑室肿瘤生长迅速。进行了第二次手术,并切除了该肿瘤。再一次,病理诊断为WHOⅠ级CPP。作者介绍了这一罕见病例,并讨论了当前的相关文献。
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