Hemangioblastoma

A hemangioblastoma is a rare noncancerous (benign) tumor. It starts in the cells that form blood vessels in your brain, spinal cord or the tissue at the back of your eye (retina). Although these tumors aren’t cancer, they can cause issues if they grow large enough to press on nearby tissues.

Hemangioblastoma types

Hemangioblastoma (huh-man-jee-ow-bla-STOW-ma) types refer to the part of your body that a tumor affects:

• Cerebellar hemangioblastoma: The tumor is in the cerebellum in your brain. Providers may say it’s a type of brain tumor. The American Brain Tumor Association reports hemangioblastomas make up 2 out of 100 brain tumors.
• Retinal hemangioblastoma: This is a rare form of hemangioblastoma that forms in your retina. It affects 1 out of 73,000 people.
• Spinal cord hemangioblastoma: This disease makes up 2 out of 100 spinal tumors. It typically develops in the middle and top sections of your spine.

Symptoms of this condition

Hemangioblastoma symptoms vary depending on the tumor’s location. For example, a tumor in your cerebellum in your brain may cause symptoms like:

• Balance problems
• Headaches
• Nausea or vomiting

A tumor in your retina may cause retinal detachment or loss of vision. Symptoms may be:

• Eye pain and swelling
• Flashes of light, floaters or darkening of your vision

A tumor on your spinal cord may cause symptoms like:

• Constipation
• Difficulty when you poop
• Leaking pee if you’re having issues controlling your bladder
• Numbness or tingling sensation in your arms or legs

Hemangioblastoma causes

Most tumors develop for unknown reasons. But research shows the genetic disorder Von Hippel-Lindau disease (VHL) accounts for about 1 in 4 cases. People with VHL have an 8 out of 10 chance of developing a hemangioblastoma in their brains or spines. Up to 6 out of 10 people with VHL may have retinal hemangioblastoma.

Complications of this condition

Without treatment, a hemangioblastoma may cause complications like:

• Fluid buildup in your brain if a tumor blocks the flow of fluid that cushions your brain
• Permanent weakness or numbness if a tumor puts pressure on your spinal cord
• Loss of vision if it affects your retina

People with VHL may have tumors that come back after treatment.

How doctors diagnose a hemangioblastoma

Your healthcare provider will ask you about your symptoms, health and family history. They may do tests like:

• An angiography, a special X-ray that gives healthcare providers a clear look at a hemangioblastoma
• A CT scan
• An MRI

How is this condition treated?

Your healthcare provider may do surgery to remove the hemangioblastoma. Other treatments are:

• Active surveillance: Hemangioblastomas are slow-growing tumors. If you don’t have symptoms, your provider may recommend delaying surgery or other treatment. You’ll have regular follow-up appointments so your provider can check for changes.
• Medication: You may receive belzutifan (Welireg®) if you have VHL that causes a hemangioblastoma.
• Photodynamic therapy: This is treatment for a retinal hemangioblastoma.
• Radiation therapy: This treatment uses energy to shrink or destroy the tumor.

When should I seek care?

A hemangioblastoma can affect your body in several different ways. In general, you should talk to your healthcare provider if you have:

• Balance issues
• Vision changes, like floaters or a shadow that blocks your vision
• Numbness

What can I expect if I have this condition?

Surgery that successfully removes the tumor can cure a hemangioblastoma. But the tumor may cause long-term issues like nerve damage or vision loss. Your healthcare provider will explain what you can expect, given your situation.

A hemangioblastoma can come back. You’ll have regular checkups so your healthcare team can look for signs of a new tumor. Regular checkups increase the chance your team will find and treat the new tumor while it’s still small and easier to treat.

What is the difference between a hemangioma and a hemangioblastoma?

A hemangioma is a benign (noncancerous) growth that affects your skin. A hemangioblastoma is a tumor that develops in your brain, spinal cord or retina.