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Posterior fossa ependymoma

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Ataxia and headaches.

Patient Data

Age: Young adult

Brain

mri

MRI of the brain demonstrates a large heterogeneous mass centered in the fourth ventricle and herniating out of the left foramen of Luschka. It is mostly isointense to the adjacent cerebellum on T1 weighted images, hyperintense on T2 (with areas of higher signal / cystic change), and without significant restricted diffusion. Post contrast images demonstrate vivid but heterogeneous contrast enhancement.

There is no surrounding edema, and although the fourth ventricle is effaced there are no features to suggest significant hydrocephalus on the images provided. 

Case Discussion

This patient went on to have a posterior fossa craniotomy and resection of the tumor. 

Histology

Sections show an ependymal tumor showing perivascular pseudorosette formation. Localized foci of recent tumor necrosis are identified. Vascular proliferation is not seen. Increased mitotic activity is not observed. 

FINAL DIAGNOSIS: Ependymoma containing foci of recent tumor necrosis.

Note: as molecular profiling was not carried out in this case, it would have the diagnosis of posterior fossa ependymoma not otherwise specified (NOS). 

Discussion

This case demonstrates fairly typical imaging features of a posterior fossa ependymoma. Tumor necrosis, which was focally noted in this ependymoma, is one histologic parameter used for grading ependymomas. There were no other histologic features associated with aggressive biologic behavior in this sample. It should be noted that the correlation between tumor necrosis and biologic behavior is not as stringent in ependymomas as in diffusely infiltrating astrocytomas.

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