Supratentorial ependymoma

Case contributed by Assoc Prof Frank Gaillard

Presentation

Headache and seizure.

Patient Data

Age: 70 years
Gender: Male
CT

An intra-axial cystic mass is seen in the right temporal lobe. This demonstrates enhancing rim and enhancing nodular septations. Mass effect with sulcal effacement in the right cerebral hemisphere. Effacement of the right lateral ventricle. Midline shift to the left with right subfalcine and uncal herniation. Cerebellar tonsils are anatomically positioned. No intracranial hemorrhage or cerebral surface collection.

MRI

Contrast not administered due to developing acute renal impairment.

A right temporal intra-axial multiloculated cystic appearing mass with the nodular and linear bands of tissue, shown to enhance on CT, are present. The surrounding FLAIR hyperintensity extends to the cortex. Local mass-effect with right hemispheric sulci effacement, displacement and compression of the right lateral ventricle, and midline shift. Uncal herniation distorts the right midbrain. Part of the nodule demonstrates susceptibility and high CBF on ASL. The wall is also partially lined by susceptibility. No fluid-fluid level. Spectroscopy demonstrates only the occasional voxel with elevated choline. No other lesion is identified apart from several small scattered foci of FLAIR hyperintense in the supratentorial white matter.

Conclusion: Unusual appearing lesion. In this age group an atypical appearing GBM or an unusually large solitary metastasis are statistically most common, however, would usually have different appearances. Alternative diagnoses that more commonly would have this appearance, but more commonly occur in a younger age group, include a PXA, ganglioglioma or supratentorial ependymoma.

Case Discussion

The patient went on to have a resection. 

Histology: 

Sections show a markedly cellular glioma with a discrete border with adjacent gliotic brain. The tumor cells are predominantly monomorphic malignant cells oval nuclei, fine nuclear chromatin and pale eosinophilic cytoplasm. The cells are arranged into sheets as well as palisading along the edge of cystic spaces. There are prominent ependymal canals and pseudorosettes. Mitoses are frequent (5 per 10 high power fields). There is abundant non-palisading necrosis and microvascular proliferation is seen. Scattered foci showing marked cytological atypia and present.

Immunohistochemistry:

  • EMA positive
  • CD99 positive
  • M2A positive
  • S100 positive
  • GFAP positive
  • IDH1 R132H negative
  • Ki67 index = ~30%
  • P53 positive
  • ATRX - positive (not mutated)

FINAL DIAGNOSIS: Anaplastic supratentorial parenchymal ependymoma (WHO Grade III).

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Case information

rID: 71974
Published: 2nd Nov 2019
Last edited: 29th Mar 2020
Inclusion in quiz mode: Included

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