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两例婴儿室管膜下巨细胞星形细胞瘤的基底节部位。

Basal ganglia location of subependymal giant cell astrocytomas in two infants.

机构信息

Department of Pediatrics, Division of Pediatric Neurology, Acibadem University, Istanbul, Turkey.

出版信息

Pediatr Neurol. 2010 Feb;42(2):157-9. doi: 10.1016/j.pediatrneurol.2009.09.008.

Abstract

Subependymal giant cell astrocytomas are benign tumors that constitute one of the primary features of tuberous sclerosis. Two infants with tuberous sclerosis had very unusual subependymal giant cell astrocytomas, confirmed on biopsy in one of the infants. In both cases, contrast-enhanced cranial magnetic resonance imaging suggested a calcified intra-axial mass with diffuse basal ganglia involvement extending into the lateral ventricle. Computed tomography confirmed calcification in both cases. The first patient had right temporal lobectomy for intractable epilepsy. Biopsy of the basal ganglia lesion in that case suggested subependymal giant cell astrocytoma. In infants, subependymal giant cell astrocytomas can present with unusual morphology and may feature diffuse basal ganglia involvement and severe calcification.

摘要

室管膜下巨细胞星形细胞瘤是良性肿瘤,是结节性硬化症的主要特征之一。两名结节性硬化症婴儿患有非常罕见的室管膜下巨细胞星形细胞瘤,其中一名婴儿的活检结果得到证实。在这两种情况下,增强颅磁共振成像提示有钙化的颅内实质性肿块,弥漫性基底节受累延伸至侧脑室。计算机断层扫描在两种情况下均证实了钙化。第一个患者因难治性癫痫进行了右颞叶切除术。对该病例的基底节病变进行活检提示室管膜下巨细胞星形细胞瘤。在婴儿中,室管膜下巨细胞星形细胞瘤可能表现出不寻常的形态,可能具有弥漫性基底节受累和严重的钙化。

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