Presentation
Headache.
Patient Data
There is a lesion of heterogenous signal intensity arising from the cerebellar vermis extending more to the left side. It is predominantly solid with rim-enhancing cystic component at its inferior aspect. Multiple flow voids at the posterior margin of the lesion suggestive of vascular origin. Severe posterior mass effect at the cerebral aqueduct and fourth ventricle with associated hydrocephalus and transtentorial edema. There is flattening of the pons and extension of the cerebellar tonsils into the foramen magnum. There is no thickening of enhancement of the dura or diffusion restriction.
Conclusion: demographics and appearance of the mass, particularly the prominent flow-voids, suggests a hemangioblastoma as the most likely diagnosis.
Case Discussion
The patient underwent posterior fossa craniotomy with excision of the lesion.
Histology
MICROSCOPIC DESCRIPTION Sections of the cerebellum show a moderately cellular tumor with scattered stromal cells in a background of many blood vessels of varying sizes. The tumor cells form aggregates. They have enlarged nuclei with hyperchromasia and foamy vacuolated cytoplasm. Mitoses are inconspicuous. There is no necrosis. The tumor cells are focally inhibin positive. CAM5.2 is negative.
FINAL DIAGNOSIS: hemangioblastoma (WHO Grade I)
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