Glioblastoma, IDH-wildtype - pediatric

Case contributed by Ryan Thibodeau
Diagnosis certain

Presentation

Progressively worsening diplopia and right-sided weakness associated with headaches, nausea, and vomiting.

Patient Data

Age: 14 years
Gender: Male

There is a well-circumscribed, expansile lesion which appears to be centered at the lateral aspect of the left lateral ventricle that displays margins of heterogeneous, thickened enhancement and central areas of cystic necrotic changes.

There is restricted diffusion within the splenium of the corpus callosum. Note is also made of T2 prolongation along the ependymal lining of both lateral ventricles, likely reflecting transependymal flow of CSF secondary to hydrocephalus.

There is focal midline shift to the right, without evidence of uncal herniation.

Case Discussion

This is a wildtype IDH-1 (non-mutated) glioblastoma. Given its proximity to the lateral ventricle and its imaging features, a choroid plexus tumor was initially considered for this pediatric patient.

The patient then underwent a left parietal craniotomy for resection of the tumor and gross pathology revealed an irregularly-shaped tan-gray soft tissue mass.

Histologically, the tumor was composed of normal choroid plexus and edematous brain tissue with areas of necrosis. There were rare, atypical cells. The tumor was positive for GFAP, and negative for EGFR and CD34. The IDH-1 was found to be non-mutated. There was elevated Ki-67 reaching >40% in hot spots. There was no significant mesenchymal production on Reticulin and Trichrome stains. The infiltrative nature is highlighted on Neurofilament stain. Stain for P53 labels about 15% of the tumor cells. Analysis was negative for the 1P/19Q co-deletion, EGFR amplification on FISH, and MGMT promoter methylation.

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