A hemangioblastoma is a benign and slow-growing tumor in the brainstem or spine. It’s a well-defined growth that forms in the lining of your blood vessels. A hemangioblastoma does not harm healthy tissue nearby and can be treated with surgery or focused radiation.
Hemangioblastoma Overview
Hemangioblastomas grow in the blood vessels of the brain, spinal cord, and retina. These tumors can be caught early and removed before they grow too big. The tumors are benign. That means they will not become cancerous. But they can press on areas of the brain and cause various neurological symptoms.
Causes. A hemangioblastoma mostly grows without an obvious cause. This makes them impossible to predict. Recurring hemangioblastomas may signal an underlying genetic condition such as von Hippel-Lindau (VHL) disease.
A hemangioblastoma occurs as a result of VHL. It is an inherited genetic condition. Around 20% to 25% of hemangioblastoma cases are caused by VHL. It affects 1 in 36,000 people.
Hemangioblastomas are usually single tumors. Multiple tumors can appear. But that typically happens only in people with VHL.
VHL causes a mutation of the VHL gene. This leads to the growth of benign tumors in many different parts of the body. The condition is diagnosed at an early age. There is no cure or prevention for VHL. The primary treatment is surgically removing tumors.
Risks and complications. A hemangioblastoma arises randomly. The people most at risk are those with VHL. The tumors are benign and may not need treatment. Yet they can start causing certain symptoms if they grow too large.
Hemangioblastomas often grow repeatedly in people with VHL. They are the cause of death for 82% of that group.
Symptoms of a Hemangioblastoma
Large tumors put pressure on your brain and spine. A hemangioblastoma tends to press against the cerebellum and brainstem. Common symptoms of one include:
- Headache
- Loss of coordination
- Imbalance
- Nausea
- Vomiting
The cerebellum is a small area of the brain next to the brainstem. It is also called the “little brain.” Your cerebellum manages your body’s balance, movement, and coordination. Putting pressure on the cerebellum can impair your sense of movement.
Pressure on the brainstem and spinal cord may cause:
- Loss of motor function
- Muscle weakness
- Numbness in your extremities
- Impaired bowel function
- Impaired bladder function
Cerebellar edema. A hemangioblastoma can cause enough pressure in the brain to cause swelling. Cerebral edema is when swelling occurs due to excess fluid in your brain tissue. This can be life-threatening.
Hemangioblastoma Diagnosis
There are three main methods for diagnosing a hemangioblastoma.
Computed tomography (CT) scan. This method uses a series of X-rays taken from multiple angles around your body. A computer processes the images to create cross-sectional images of your bones, blood vessels, and soft tissue. A hemangioblastoma will appear on these X-rays.
Magnetic resonance imaging (MRI). This method uses a magnetic field and radio waves to make detailed images of your organs and tissues.
Cerebral angiography. This method involves injecting a contrast dye into your bloodstream. An angiography causes the bloodstream to show up on an X-ray and reveals abnormalities in your blood vessels.
Hemangioblastoma Treatment
Surgery is the standard treatment option for a hemangioblastoma. This typically cures the condition unless the entire tumor can’t be removed. Your doctor may use some of the diagnostic methods mentioned above to monitor your recovery after surgery.
There’s a chance surgery can eliminate the problem entirely for people with VHL. Only about 25% of people receiving surgery for a hemangioblastoma ever require another surgical treatment.
Stereotactic radiosurgery (SRS). This type of radiation therapy may be used if surgery may result in complications. It involves using a concentrated dose of radiation to eliminate the hemangioblastoma.
The beams of radiation stop blood flow to the tumor. This causes the tumor to shrink over several months. The goal of the procedure is to prevent the tumor from growing.
SRS uses precise, focused beams of radiation to target tumors. Using 3D imaging, the beams deliver concentrated radiation to the affected area without significantly harming the surrounding tissue.
SRS of the brain and spine usually only requires a single session. The chance of risk or complications is low. There are very few side effects to SRS. Most of them are temporary.