History: An 8-year-old girl presents with persistent headaches for the past several weeks and nausea and vomiting for past several days.
A CT scan of the head was obtained in the emergency department.
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.
Postcontrast sagittal T1-weighted image is shown below.
- 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.
- Diffuse intrinsic pontine glioma
- Tectal glioma
- Neurofibromatosis type 1
- Osmotic demyelination syndrome
- Acute disseminated encephalomyelitis
- 神经纤维瘤病 1型
Diagnosis: Diffuse intrinsic pontine glioma
Diffuse intrinsic pontine glioma
- 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.
- 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.
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.
- 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).