Posterior fossa ependymoma

Case contributed by Jini P Abraham
Diagnosis certain

Presentation

History of sudden onset headache and vomiting. No seizures.

Patient Data

Age: 30 years
Gender: Male
mri

T2 hypointense signal in the cerebral aqueduct and fourth ventricle, causing expansion. SWI shows areas of blooming. Post-contrast study shows no enhancement.

Bilateral lateral and 3rd ventricles are dilated (Evans index 0.4). SWI blooming noted in the ventricular system - suggestive of intraventricular hemorrhage with obstructive hydrocephalus. The convolutional markings on the skull and no trans ependymal edema indicates raised intracranial pressure.

Thinning and elevation of corpus callosum noted. Diffuse effacement of sulcal spaces of bilateral cerebral hemispheres noted. Cerebellar tonsillar herniation noted.

Area of diffusion restriction noted in right high frontal lobe – acute infarct.

He underwent midline suboccipital craniotomy and decompression under general anesthesia.

Intraoperative findings

Tumor arising from brainstem with a dorsally exophytic component. Soft to firm, grayish red, highly vascular tumor. The exophytic portion was excised and the brainstem portion was left behind.

Histopathology

MICROSCOPY: The section shows tumor tissue composed of small uniform cells. The cells have scant cytoplasm with uniform round to oval nuclei, and finely granular chromatin in a fibrillary matrix. Capillary proliferation with myxoid degeneration noted.

Features are consistent with ependymoma - WHO grade 2.

Case Discussion

Ependymomas in children arise infratentorial, frequently fill the fourth ventricle and can extend through the foramen of Luschka or Magendie to the dorsal aspect of the spinal cord.

Ependymomas in adults occur in the spinal cord commonly and intracranial involvement is rare. These tumors show areas of hemorrhage and calcification on imaging. Hydrocephalus is a common finding. There are various subtypes of ependymoma, and out of these, the posterior fossa type A - 2c subtype is said to have the best prognosis.

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