History: Toddler undergoing imaging after having a single seizure. Patient was developmentally and neurologically normal prior to presentation.
学步幼儿(1岁左右)惊厥发作一次,在此之前发育和神经系统表现正常。
CT images are shown below.
CT图像如下
MR images are shown below.
MR图像如下
CT: CT images demonstrate a mildly hyperattenuating intraventricular lobular mass arising within the lateral body of the right lateral ventricle. There is associated ventriculomegaly, indicative of at least mild hydrocephalus.
MRI: MR images confirm the presence of a well-delineated, lobulated mass in the right lateral ventricle. Mass is isointense to gray matter on T1-weighted imaging and demonstrates heterogeneous enhancement following gadolinium-DTPA. Bright periventricular signal on axial fluid-attenuated inversion-recovery (FLAIR) images likely represents mild transependymal interstitial edema due to hydrocephalus.
Differential diagnosis
Choroid plexus papilloma/carcinoma
Intraventricular meningioma
Papillary ependymoma
Diagnosis:
Choroid plexus papilloma
(WHO grade I)
表现
CT:右侧侧脑室体部内略高密度分叶状肿物,合并有脑室扩大,提示轻度脑积水。
MRI:右侧侧脑室体部边界清楚的分叶状肿物,T1WI呈与灰质相同的等信号,增强扫描不均质强化。FLAIR示侧脑室周围高信号,提示脑积水所致的室管膜周围轻度间质性水肿。
鉴别诊断:
脉络丛乳头状瘤/癌
脑室内脑膜瘤
乳头状室管膜瘤
诊断:脉络丛乳头状瘤(WHO I级)
Key points
Choroid plexus tumors (CPTs)
Choroid plexus tumors are intraventricular, papillary neoplasms derived from choroid plexus epithelium.
They are one of the more common supratentorial brain tumors in children younger than 2 years of age; they represent the most common intraventricular neoplasm in children.
86% present by 5 years of age
World Health Organization (WHO) grade I
70% of cases occur in the atrium of the lateral ventricle (left more common that right), and 20% occur in the fourth ventricle (most common site of origin in adults).
Imaging findings:
CT: Iso- or hyperattenuating intraventricular lobular mass
25% of the tumors have calcifications.
Hydrocephalus is common due to ventricular obstruction and/or cerebrospinal fluid (CSF) overproduction.
Contrast-enhanced CT: Intense, homogeneous enhancement
MRI:
T1-weighted imaging: Iso- to hypointense well-delineated, lobulated intraventricular mass.
T2-weighted imaging: Iso- to hyperintense ± internal linear and branching flow voids.
FLAIR: Bright periventricular signal due to transependymal interstitial edema.
T1-weighted imaging with contrast: Robust homogeneous enhancement; cysts and small foci of necrosis may be present. May spread via subarachnoid space.
May become anaplastic over time. Imaging alone cannot reliably distinguish between choroid plexus papillomas and carcinomas.
Treatment is total surgical resection. Five-year survival is close to 100%.
重点
脉络丛乳头状肿瘤是起源于脉络丛上皮的脑室内乳头状肿瘤。是2岁以下幼儿最常见的幕上脑肿瘤之一,是儿童最常见的脑室内肿瘤。86%的在5岁以下;肿瘤为WHO I级;70%发生在侧脑室(左侧多于右侧),20%发生在四脑室(为成人好发部位)。
影像表现:
CT:侧脑室内等或稍高密度分叶状肿物,25%可见钙化,常合并脑积水(由于脑室梗阻或脑脊液过度产生);增强扫描呈明显均匀强化。
MRI:
T1WI:等或低信号,边界清楚,分叶状的脑室内肿物;
T2WI:等或高信号,瘤内可见线样和分支样流空效应;
FLAIR:脑室周围见高信号(室管膜周围间质性水肿)
增强:明显均匀强化,可见小囊变、坏死区,可沿蛛网膜下腔播散。
随着时间进展肿瘤可变成间变性,仅靠影像无法明确鉴别脉络丛乳头状瘤和癌。
治疗方法为完全手术切除,五年生存率接近100%。
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