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Aimee双语小讲堂 第十一讲:室管膜下瘤

 忘仔忘仔 2022-03-06

Aimee   上海某医院

59-year-old woman with incidental brain tumor 

59岁女性,偶然发现颅内肿瘤 

History: A 59-year-old woman with a history of an incidental finding of a brain tumor during an examination for labyrinthitis 18 years ago. The patient returned after that time for further investigation.

病史:59岁女性,18年前因内耳炎就诊检查发现颅内肿瘤。患者目前为进一步诊治来院。

Axial noncontrast CT image, sagittal T1-weighted and axial T2-weighted MR images, axial susceptibility-weighted image (SWI), postgadolinium image, sagittal 3D constructive interference in steady state (CISS/FIESTA-C) image, and short echo-time (TE) 3D multivoxel spectroscopy image are shown below. Click to enlarge.

平扫CT横断位,磁共振T1矢状位、T2横断位,磁敏感横断位,增强磁共振扫描,矢状位磁共振增强三维双激发平衡式自由稳态进动序列如下。

Images of the macroscopic specimen and hematoxylin and eosin (H&E) stain also are shown.

大体标本图像和HE染色图像如下

Findings and diagnosis

Findings

Noncontrast CT axial image shows a predominantly hypodense mass in the temporal horn of the right ventricle. Note, there are tiny hyperdense foci (arrow), probably related to previous microhemorrhages (A). Sagittal T1-weighted MR image confirms a lobulated, well-defined intraventricular. The mass is primarily hypointense to gray matter and causes expansion of the temporal horn of the right lateral ventricle (B). Axial T2-weighted MR image shows the mass is heterogeneously hyperintense, and there is hyperintensity in the adjacent brain parenchyma of the temporal lobe, that might represent an edema (or infiltrative) component (C, arrow). Axial susceptibility-weighted image (SWI) shows small hypointense foci inside the lesion, corresponding to microhemorrhages (D). There was no or minimal enhancement postgadolinium (E). Sagittal 3D-CISS (FIESTA) images show a heterogeneous high-signal-intensity solid lesion, with cystic components within the lesion (F, arrow). Short echo time (TE) 3D multivoxel spectroscopy demonstrated the N-acetylaspartate (NAA) level partially decreased, due to neuronal viability loss, normal peak of choline, and an increased peak of myoinositol, meaning astrogliosis, compared with the normal left temporal lobe (G).

CT平扫横断位可见右侧脑室颞角明显低密度肿块。注意病灶内细小高密度灶(箭头),可能与微出血相关(A)。磁共振T1矢状位可见脑室内分叶状、边界清晰占位。病变相对灰质呈低信号,右侧脑室颞角扩张(B)。T2横断位可见肿块不均匀高信号,邻近颞叶脑实质呈高信号,可能是水肿(或浸润)成分(C,箭头)。磁敏感横断位可见病灶内细小低信号灶,说明存在微出血(D)。注入对比剂后未见明显/轻微强化(E)。磁共振增强三维双激发平衡式自由稳态进动序列矢状位可见不均匀高信号实性病灶,内部可见囊性成分(F,箭头)。磁共振波普可见NAA峰降低,是因为神经元丢失引起。Cho峰未见明显异常。肌醇峰升高,说明较对侧正常颞叶,患侧颞叶的胶质细胞增生。

The macroscopic specimen obtained at surgical resection correlates with the imaging findings: a lobulated mass, with heterogeneous consistency, containing small cysts (H, arrows). Histological slices with H&E staining reveal a pluricellular neuroepithelial neoplasm, characterized by small clusters of glial cells, with isomorphic vesicular nuclei (I).

大体标本为手术切除病灶:不均质分叶肿块,内见小囊肿(H,箭头)。组织学标本HE染色后可见多细胞神经上皮新生物,由小簇状胶质细胞组成,伴同形的小泡细胞核。

Differential diagnosis

鉴别诊断

· Subependymoma

· Ependymoma

· Neurocytoma

· Giant cell subependymal astrocytoma

· 室管膜下瘤

· 室管膜瘤

· 神经细胞瘤

· 巨细胞室管膜下星形细胞瘤

Diagnosis: Subependymoma

诊断:室管膜下瘤

Key points

Subependymoma

· The reported case illustrates an intraventricular mass at an unusual location of the lateral ventricles: the temporal horn.

· 已报道的病例提示病灶位于侧脑室不常见位置:颞角。

· Subependymomas are usually single lesions in the fourth and lateral ventricles in patients frequently asymptomatic. When the lesion is greater than 2.0 cm, it can cause obstruction of cerebral spinal fluid circulation, causing hydrocephalus.

· 室管膜下瘤通常为第四和侧脑室内单发病灶,患者通常无症状。当病灶大于2.0公分,可引起脑脊液流动阻塞,继发脑积水。

· CT shows a well-circumscribed macrolobulated intraventricular mass, that is heterogenous, hypo/isodense to brain parenchyma. Usually, there is no periventricular extension and no contrast enhancement.

· CT可见脑室内边界清晰的浅分叶肿块,密度欠均匀,相对脑实质呈低/等密度。通常不累及脑室周围,病灶无强化。

· MR imaging shows an hypo/isointense lesion on T1-weighted imaging and hyperintense on T2-weighted imaging. Cystic degeneration may occur. If there is contrast enhancement, it is typically tenuous and heterogeneous.

· 磁共振T1图像可见低/等信号、T2高信号病灶。可见囊性变。注入对比剂后呈轻度均匀强化。

· Histological features include low cellular density and small clusters of glial cells, dispersed in a loose fibrillary matrix, near the ependymal lining, considered a benign neuroepithelial neoplasm (World Health Organization [WHO] grade I). Recurrence is very rare.

· 组织学特征包括低细胞密度,小簇状的胶质细胞,分散于纤维基质中,邻近室管膜,为良性神经上皮细胞肿瘤(WHO I级)。病灶极少复发。

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