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Hemangioblastoma

Background

Hemangioblastomas are benign tumors of the surface of the brain, similar to meningiomas in many ways, but with a greated tendency to recur.

Hemangioblastomas are occasionally associated with an inherrited disorder called Von Hippel-Lindau disease, where it is associated with other tumors such as retinal angiomas, pancreatic cysts, pheochromocytoma and renal cell carcinoma.  Von Hippel-Lindau patients typically develop multiple hemangioblastomas.

Evaluation

Common symptoms include headache, loss of coordination, imbalance, nausea and vomiting. with spinal hemangioblastomas, muscle weakness, numbness constipation and incontinence can occur.  Because the flow of CSF can be cut off, symptoms can develop suddenly, but a long course of symptoms is more typical.

Imaging

CT scan of the brain will show most hemangioblastomas, but hemangioblastomas of the spine require an MRI.  

Treatment

Surgery: Surgical resection is effective at removing the tumor in most cases. Surgery usually requires an open craniotomy.

Cyberknife Radiosurgery: for spinal lesions, radiosurgery has been a standard treatment for hemangioblastoma for many year. A recent report from the UCLA David Geffen School of Medicine showed a 90% four year local control for these lesions.

For this reason, radiosurgery is also used for unresectable hemangioblastomas of the brain.

In unresectable brain hemangioblastomas, four year local control after radiosurgery was 71%. Given that these tumors are likely larger than those of patients who were surgical cancdidates, this rate of control is impressive. Radiosurgery is a reasonable alternative for the treatment of hemangioblastomas in patients who do not wish to undergo brain surgery.