Presentation
Two week history of intermittent gait instability, confusion, and headaches. One week history of severe nausea and vomiting.
Patient Data
There is an intensely enhancing mass in the left cerebellar hemisphere with high vascularity, including numerous vessels in and around the mass. There is a focal hypodensity and presumed nonenhancement in the anterior aspect of the mass. There is susceptibility artifact within the mass.
There is large amount of surrounding edema with severe compression and displacement of the fourth ventricle. The supratentorial ventricles are moderately dilated and there is periventricular edema indicating transependymal CSF flow.
Case Discussion
This is a hemangioblastoma, CNS WHO Grade 1.
Initially the patient underwent biopsy which intraoperatively revealed cerebellar tissue with neoplastic cells suspicious for hemangioblastoma. This was followed by left paramedian sub-occipital craniotomy for direct tumor embolization and resection with placement of a right frontal external ventricular drain (EVD).
Gross pathology revealed friable, irregular tan-pink, lobulated soft tissue. Histopathology was consistent with cellular proliferation composed of closely-packed thin-walled vessels within stromal neoplastic cells with bland nuclei and clear or foamy cytoplasm. In addition, a portion of the tumor cells demonstrated enlarged nuclei with eosinophilic granular cytoplasm. Immunohistochemical analysis demonstrated positivity for CD56 and inhibin. Further molecular testing revealed the tumor was negative for EMA and PAX8. Overall, these pathologic findings were consistent with hemangioblastoma (CNS WHO grade 1).
Post-operatively, the patient had no complications and the EVD was removed after one week. Patient completed short stay inpatient rehabilitation and saw neurosurgery as an outpatient.
Co-author:
Trevina Soliman
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