Hemangioblastoma

Case contributed by A.Prof Frank Gaillard

Presentation

Intermittent headache and nausea.

Patient Data

Age: 45 years
Gender: Male
MRI

Single lesion identified in the right side of the posterior fossa located peripherally is probably intra-axial but does abut the dura. This measures 2.3 cm in size and shows avid enhancement, associated with vasogenic edema in the adjacent cerebellum. Prominent flow voids are noted at the periphery of the lesion, with some signal loss due presumably to prior hemorrhage. MR spectroscopy demonstrates prominent lipid peak. This mass effect pushes on the inferior aspect of the medulla oblongata and partially effaces the fourth ventricle with obstructive hydrocephalus and transependymal edema.

Low-lying cerebellar tonsils consistent with an acquired chiari type I. Otherwise the brain is unremarkable. No white matter lesions are seen as brain. No extra-axial collection. 

Conclusion:

In this age group, particularly in the absence of known systemic malignancy, a posterior fossa mass with prominent flow voids most likely represents a hemangioblastoma. Less likely alternative diagnoses include solitary metastasis. 

DSA (angiography)

Technique: After informed consent and under sterile technique, the right CFA was accessed using a 6Fr sheath, and selective injections were made into the common carotid and vertebral arteries bilaterally.

Findings: The tumor is markedly hypervascular, with rapid arteriovenous shunting, with supply from inferior hemispheric branches of the large right PICA. No significant supply was identified from the left PICA. Draining veins correlate with those demonstrated on the stereotactic post contrast MRI. No other significant supply. No significant stenoses, fibromuscular disease, or intracranial aneurysms. No other hypervascular tumor is identified.

Embolization:  A 5Fr guiding catheter was placed in the right vertebral artery, a SL 10 microcatheter navigated over a Synchro soft microwire into the super selective branch directly supplying the tumor. 250-350 \XB5\ PVA prolonged injection with almost complete obliteration of the hypervascular stain, with preservation of the major branches of PICA. The procedure was uncomplicated, no new symptoms or signs developed.

Final angiographic runs demonstrate complete obliteration of the tumor stain, with the exception of a small amount seen inferiorly, with no persistent arteriovenous shunting.

Case Discussion

Following embolization the patient underwent a craniotomy and resection. 

Histology

MICROSCOPIC DESCRIPTION:

Sections of the cerebellum show scattered stromal cells in a background of many capillary-sized blood vessels. There are areas of hemorrhage. The tumor cells show mildly enlarged nuclei with hyperchromasia and foamy vacuolated to pale eosinophilic cytoplasm. Mitoses are inconspicuous. There is no necrosis. Some of the blood vessels contain foreign body material, consistent with preoperative embolization. The cerebellar parenchyma shows reactive gliosis and hemosiderin deposition. The tumor cells are inhibin positive. They are CAM5.2 and CD10 negative. The features are those of hemangioblastoma. There is no evidence of malignancy.

DIAGNOSIS:

Hemangioblastoma (WHO Grade I).

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Case information

rID: 33046
Published: 29th Dec 2014
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included

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