Ependymoma

Case contributed by Dr Bruno Di Muzio

Presentation

Headache. CT brain showing hydrocephalus.

Patient Data

Age: 50 years
Gender: Male
MRI

MRI Brain

There is a solid frond-like mass occupying and expanding the inferior aspect of the fourth ventricle, with the tumour extending through the right foramen of Luschka and inferiorly through the foramen of Magendie and causing mild hydrocephalus. The caudal extent of the tumour reaches the level of C1-C2. This tumour has low T1 signal and increased T2/FLAIR signal, with heterogeneous though not intense contrast enhancement, and multiple small susceptibility foci consistent with microhaemorrhages. It does not cause restricted diffusion compared to the brain parenchyma. There is a mass effect with anterior displacement of the medulla oblongata on the right. Associated cerebellar descent below the foramen magnum. The remainder of the brain appears normal, there is no transependymal oedema surrounding the hydrocephalus. No evidence of leptomeningeal/CSF seeding within the brain or imaged upper cervical spine.

Pathology

Sections showing moderately cellular tumour with a fibrillary background forming prominent perivascular pseudorosettes and focal ependymal canals.

Case Discussion

Imaging characteristics are most consistent with an ependymoma, although the location of the tumour is less typical in this age group. A choroid plexus papilloma is felt less likely given the not so vivid enhancement. 

 

MICROSCOPIC DESCRIPTION: 1-2. Sections show a moderately cellular tumour with a fibrillary background forming prominent perivascular pseudorosettes and focal ependymal canals. No true ependymal rosettes are seen. Tumour cells demonstrate mildly enlarged elongated nuclei and small nucleoli. No mitoses, necrosis or microvascular proliferation are seen. Immunohistochemically tumour cells stain:

  • GFAP+
  • ATRX+
  • IDH1-
  • p53-
  • EMA-.
  • Ki67 proliferation index is approximately 5%.

DIAGNOSIS: 1-2. Brain, 4th ventricle lesion: Ependymoma (WHO Grade II)

Slide images courtesy of the RMH Pathology Department. 

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Case information

rID: 55003
Case created: 14th Aug 2017
Last edited: 21st Aug 2017
Tag: rmh
Inclusion in quiz mode: Included

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