【双语病例】脑室内多形性胶质母细胞瘤(GBM)

History

A 62-year-old man with weakness and dizziness for the past two weeks.

62岁女性，头晕乏力2周。

1.There is a mass primarily located in the frontal horn of the right lateral ventricle.

肿块位于右侧侧脑室前角。

True

False

2.There is hydrocephalus.

该患者有脑积水。

True

False

3.Which of the following are the most common intraventricular frontal horn masses?

下列哪些病变好发于侧脑室前角？

A. Central neurocytoma 中枢神经细胞瘤

B. Ependymoma/subependymoma 室管膜瘤/星形细胞增生性室管膜瘤

C. Lymphoma/metastasis 淋巴瘤/转移瘤

D. Meningioma 脑膜瘤

E. Both A and D

F. All of the above

MR images

Brain MR images without and with contrast are shown below.

4.MRI confirms the mass to be primarily intraventricular.

MRI可以证实肿块位于脑室内。

True

False

5.Imaging characteristics are most consistent with a benign intraventricular neoplasm.

影像学特点符合脑室内良性肿瘤。

True

False

6.Which of following imaging findings describes this mass?

下列哪项是对该病变合适的描述？

Predominantly heterogeneously enhancing and necrotic mass.

肿块不均匀强化，其内可见坏死区。

There is extension into the frontal lobes and corpus callosum.

肿块向额叶及胼胝体生长。

There is ependymal enhancement.

可见室管膜强化

All of the above describe the mass.

以上都是。

7.Which of the following is the most likely diagnosis?

最合适的诊断是下列哪项？

Meningioma 脑膜瘤

Ependymoma/subependymoma 室管膜瘤/星形细胞增生性室管膜瘤

Lymphoma 淋巴瘤

Central neurocytoma 中枢神经细胞瘤

None of the above

Additional MR images

Brain MR images with perfusion and spectroscopy . Click to enlarge.

8.Findings on perfusion and spectroscopy suggest a high-grade neoplasm.

灌注加权像及MRS提示病变为高级别肿瘤。

True

False

9.Which of these less common intraventricular masses is the likely diagnosis?

下列脑室内少见肿瘤中，哪项诊断更准确？

Glioblastoma 胶质母细胞瘤

Oligodendroglioma 少突胶质细胞瘤

选择题答案：

1. True
2. TrueObstruction is at the level of the foramen of Monro.

   梗阻部位位于孟氏孔水平。

3. All of the above
4. True
5. False
6. All of the above describe the mass.
7. None of the aboveThe necrotic appearance of the mass with extension into the frontal lobes and corpus callosum and ependymal enhancement are not commonly seen with these diseases

   肿块可见明显坏死、向额叶及胼胝体生长、室管膜强化等征象，而这些征象在以上病变中很少出现。

8. TrueThere is increased relative cerebral blood volume throughout the mass. Spectroscopy shows a neoplastic pattern with elevation of the choline to creatinine ratio, depression of the N-acetyl aspartate (NAA) peak, and an inverted/elevated lactate peak.

   MR灌注示肿块的CBV增加。MRS示Cho/Cr比值升高，NAA峰降低，Lac峰倒转/升高，符合肿瘤改变。

9. GlioblastomaGiven aggressive/infiltrative appearance and ependymal enhancement, glioblastoma is highly favored.

   由于病灶呈浸润性生长、室管膜强化，考虑胶质母细胞瘤可能性大。

Findings and Diagnosis

Findings

• CT head: Large heterogenous intraventricular mass causing mass effect, midline shift, and hydrocephalus.脑室内巨大不均质肿块，周围占位效应明显，伴有中线移位和脑积水。
• MRI brain:
  • Large necrotic heterogenously enhancing mass centered in the right frontal horn with intra- and extraventricular extension.肿块位于右侧侧脑室前角，向脑室内外生长，内可见坏死区，增强扫描不均匀强化。
  • Ependymal enhancement in the right frontal horn consistent with neoplastic spread.右侧脑室内室管膜强化提示肿瘤播散。

DiagnosisIntraventricular glioblastoma（GBM） multiforme脑室内多形性胶质母细胞瘤

Intraventricular glioblastoma multiforme

• Rare — primarily occurs in the frontal horn and body of the lateral ventricle.原发于侧脑室前角和体部的胶质母细胞瘤临床罕见。
• Intraventricular GBMs have the typical imaging characteristics of intraparenchymal, high-grade gliomas, including contrast enhancement and, at times, inhomogeneity and infiltrative, irregular borders.脑室内GBM的典型表现与脑实质的GBM类似，包括增强扫描明显强化、肿瘤内部不均质、浸润性生长、边界不清等。

Take-home PointsAlthough rare, when an intraventricular mass displays aggressive and infiltrative characteristics, consider GBM.尽管该病非常少见，但如果遇到脑室内侵袭性肿块，呈浸润性生长，要考虑GBM的可能性。