Presentation
Two weeks occipital headache worsening when bending over.
Patient Data
Large mass laterally in the right cerebellar hemipshere. It is predominantly isoattenuating to grey matter with some internal low attenuation foci. It demonstrates moderate heterogeneous enhancement. There is no calcification. There is vaogenic edema in the adjacent cerebellar white matter.
The 4th ventricle is partially effaced. There is crowding at the foramen magnum with effacement of the 4th ventricle outflow tracts. Mild dilatation of the lateral and third ventricles in keeping with hydrocephalus. Flattening of the posterior globes in keeping with papilledema. There is tortuosity of the optic nerves and intrasellar arachnoid herniation. The findings are in keeping with raised intracranial pressure.
Lateral right cerebellar mass shows slightly increased T2w signal, reduced T1w signal and moderate heterogeneous enhancement. The solid components show restricted diffusion. No other areas of abnormal intracranial enhancement. Associated vaogenic edema and mass effect as shown on CT. The appearances are in keeping with medulloblastoma
The optic nerve sheaths are dilated with CSF signal, especially on the left. There is flattening of the posterior sclera and high FLAIR signal at the optic nerve heads, in keeping with raised intracrainal pressure and papilledema.
The patient went on to have a resection.
Histology
Desmoplastic/nodular medulloblastoma WHO Grade 4, SHH-activated, TP53 - Wild type.
Molecular data:
- SHH-activated (by immunohistochemistry and methylation profiling)
- no MYC or MYCN amplification (by array and FISH)
- TP53 - wild type (by direct sequencing).
Case Discussion
The imaging findings of intrinsic cerebellar tumor showing moderate heterogeneous enhancement and restricted diffusion in a young adult are in keeping with medulloblastoma.
There is evidence of raised intracranial pressure, including papilledema.
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