Presentation
6-month history of occipital headache.
Patient Data
Large cystic lesion located in the inferior right cerebellar hemisphere with mild surrounding parenchymal edema. There is also a vividly enhancing mural nodule at the inferolateral aspect abutting the right jugular foramen. Multiple other smaller enhancing nodules in the posterior cranial fossa.
Additionally, a mass in the right aspect of the pituitary fossa enlarges the fossa and displaces the pituitary gland and infundibulum to the left side. The cavernous segment of the right internal carotid artery is partially encased by this mass.
Conclusion:
The appearance of the cystic lesion with a vividly enhancing mural nodule in the right inferior cerebellar hemisphere is consistent with a hemangioblastoma. Multiple smaller enhancing nodules in the posterior cranial fossa are most likely hemangioblastoma as well suggesting von Hippel Lindau disease.
Lobulated mass in the pituitary fossa is an atypical location for hemangioblastoma and pituitary adenoma remains a differential diagnosis.
Case Discussion
The patient underwent a right-sided retrosigmoid craniotomy and resection of the right inferior cerebellar hemisphere lesion.
Histology
The biopsy shows cerebellar parenchyma adjacent to a 10mm vascular rich tumor with reticular architecture. The tumor comprises cells with fibrillary cytoplasm, oval nuclei and fine chromatin intermingled with CD31 positive thin-walled blood vessels. Parenchyma adjacent tumor features numerous Rosenthal fibers.
- Positive
- Inhibin
- Negative
- EMA
- AE1/AE3
FINAL DIAGNOSIS: Hemangioblastoma (WHO Grade I).
At this stage the pituitary lesion is being observed.
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