【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

History: An 8-year-old girl presents with persistent headaches for the past several weeks and nausea and vomiting for past several days.

病史:8岁女孩,持续性头痛数周,恶心、呕吐数天。

A CT scan of the head was obtained in the emergency department.

行急诊颅脑CT平扫

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

MR images

The patient was admitted for further evaluation. An MRI exam of the head was obtained. In order: axial fluid-attenuated inversion-recovery (FLAIR), coronal FLAIR, sagittal T2-weighted, and diffusion-weighted images (DWI), as well as apparent diffusion coefficient (ADC) map.

进一步行颅脑MR扫描:轴位/冠状FLAIR、矢状T2WI、DWI、ADC图。

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

Postcontrast sagittal T1-weighted image is shown below.

矢状增强图像如下所示。

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

Findings

  • CT: There is a hypodense expansile mass centered in the pons.
  • MRI: There is an expansile mass in the pons that is homogenously T1-isointense and T2-hyperintense, and it does not exhibit enhancement or restricted diffusion.

影像表现:

  • CT:脑桥为中心的低密度膨胀性肿物
  • MR:脑桥膨胀性肿物呈均匀等T1长T2信号,无强化,无弥散受限。

Differential diagnosis

  • Diffuse intrinsic pontine glioma
  • Tectal glioma
  • Neurofibromatosis type 1
  • Osmotic demyelination syndrome
  • Acute disseminated encephalomyelitis

鉴别诊断:

  • 弥漫性内生性脑桥胶质瘤
  • 顶盖胶质瘤
  • 神经纤维瘤病 1型
  • 渗透性脱髓鞘综合征
  • 急性播散性脑脊髓炎

Diagnosis: Diffuse intrinsic pontine glioma

最后诊断:弥漫性内生性脑桥胶质瘤

Key points

Diffuse intrinsic pontine glioma

弥漫性内生性脑桥胶质瘤

Background

  • Brain tumors are the most common solid pediatric tumor.
  • Brainstem gliomas are relatively common pediatric central nervous system (CNS) tumors, representing approximately 15% of primary CNS tumors in children.
    • The pons is the most common location for brainstem gliomas.
    • It accounts for approximately 80% of cases.
  • Patients with a diffuse intrinsic pontine glioma have a very poor prognosis.
    • Although only comprising 15% of primary CNS tumors (as noted above), brainstem gliomas account for the largest proportion of deaths at approximately 38%.
    • Patients with a diffuse intrinsic pontine glioma have a median survival of less than 12 months.

背景:

  • 脑肿瘤是儿童最常见的实性肿瘤
  • 脑干胶质瘤是相对常见的儿童中枢神经系统肿瘤,在儿童原发性CNS肿瘤中约占15%;桥脑是脑干胶质瘤最常见的发病部位,约占80%。
  • 弥漫性内生性脑桥胶质瘤预后差。仅占儿童原发性CNS肿瘤的15%,脑干胶质瘤的死亡率最高,约占38%;中位生存期不足12个月。

Clinical presentation

  • Diffuse intrinsic pontine glioma often presents initially as a very large mass.
  • Symptoms typically develop relatively acutely (less than one month), although more subtle symptoms may develop over a period of months.
  • The classic described triad of symptoms includes cranial nerve deficits, ataxia, and long tract signs.
  • Patients may develop signs of obstructive hydrocephalus due to compression of the fourth ventricle.

临床表现:

  • 弥漫性内生性脑桥胶质瘤常表现为较大的肿物;
  • 症状出现的相对较急(小于1个月),尽管大部分症状轻微,但可在数月内进展;
  • 典型表现“三联征”包括:颅神经麻痹、共济失调、长束体征;
  • 随病情进展,肿瘤压迫四脑室,患者可出现梗阻性脑积水征象。

Imaging findings

Diagnosis is typically made solely on the base of imaging (due to critical location), though occasionally tissue sampling is done to confirm pathology.

  • CT: Often appears as a hypodense and expansile lesion.
  • MRI:
    • Appears as an expansile, T2-hyperintense, T1-iso/hypointense mass with no/mild enhancement and typically without associated restricted diffusion.
    • Enhancement is associated with a worse prognosis.
    • Diffuse versus focal tumor (focal with better prognosis).

影像表现:

由于特定的发病部位(脑桥),典型病例可单独依据影像进行诊断,偶尔活检进行病理学证实。

CT:常表现为低密度灶,有膨胀性;

MRI:

  • 表现为膨胀性的T2WI高信号,T1WI等或低信号肿物,无强化或轻度强化,无弥散受限;
  • 有强化提示预后较差;
  • 肿瘤弥漫或局限性(局限性预后较好)。

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR


相关文献:

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

【双语病例】弥漫性内生性脑桥胶质瘤1例CT及MR

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