What Is a Hemangioblastoma?

A hemangioblastoma is a slow-growing, noncancerous tumor that occurs in the brain, spinal cord, or occasionally the retina. Although hemangioblastomas are benign and grow gradually, they can become symptomatic and cause health issues over time. 

Hemangioblastomas are relatively uncommon, accounting for 2% of all brain tumors and 2% to 10% of spinal cord tumors. Recurrent hemangioblastomas may signal that a patient has von Hippel-Lindau disease (VHL). VHL is a hereditary gene mutation that causes hemangioblastomas, cysts, and other tumors to grow.

This article will discuss the types, causes, diagnosis, and treatment of hemangioblastomas.

Types of Hemangioblastomas

Hemangioblastomas are classified based on the type of fluid they contain and the size of the vascular channels inside the tumor. They are typically defined as cystic or solid, and are further broken down into four types:

• Type 1: A simple cyst without a nodule (the rarest, at 5% of tumors)
• Type 2: A cyst with a mural nodule (60% of tumors)
• Type 3: A solid tumor (26% of tumors)
• Type 4: A solid tumor containing small internal cysts (9% of tumors)

Hemangioblastoma Symptoms

As a hemangioblastoma grows, it puts pressure on the underlying structures within the brain and spinal cord, so symptoms may depend on the location of the tumor.

Common symptoms include:

• Nausea
• Vomiting
• Loss of balance
• Headache
• Dizziness or vertigo
• Loss of coordination

If a hemangioblastoma affects the retina, loss of vision is also a common symptom.

Causes

Most hemangioblastomas have no known cause. However, once surgically or radiologically removed, they generally do not reoccur.

However, in 25% of cases, an inherited disorder called von Hippel-Lindau disease (VHL) causes the growth of hemangioblastomas and other types of tumors and cysts in the body. VHL disease is hereditary and occurs in 1 out of 36,000 people.

Diagnosis

To diagnose hemangioblastomas, physicians will perform a diagnostic workup that will likely include a medical history, physical exam, blood tests, and a neurological examination.

MRI scans, CT scans, and spinal angiography are tests used to diagnose a hemangioblastoma and other tumors in the body.

• Magnetic resonance imaging (MRI): A diagnostic tool that utilizes magnets and radio waves to take pictures of anatomy and physiology in the body. MRIs of the brain and spinal cord can help locate tumors and surrounding structural damage that a hemangioblastoma may cause.
• Computed tomography (CT) scan: A CT scan is a type of diagnostic test that uses a series of X-rays from multiple body angles to create cross-sectional images (slices).
• Spinal angiogram: Spinal angiography is a diagnostic imaging technique that helps visualize the inside of the blood vessels that may be serving the tumor.

Treatment

Many patients undergo surgery to remove hemangioblastomas that become problematic. However, in some cases, they may be located in inoperable areas. Fortunately, there are newer technologies that make hemangioblastoma surgery safer and more effective.

The two main ways to treat a hemangioblastoma include:

• Surgery: Since hemangioblastomas are benign and slow-growing, surgical excision is usually the most common option. If surgery is too risky because the tumor is in an inoperable area, or there is a risk of compressing healthy tissue during excision, then an alternative option like radiation therapy may need to be considered.
• Radiosurgery or radiation therapy: If it is too risky to perform surgery, radiosurgery (a form of radiation therapy) is another option for hemangioblastoma treatment. The most common type of radiosurgery for hemangioblastomas is stereotactic radiosurgery. Stereotactic radiosurgery involves targeted, large-dose radiation directed at a tumor. It is a viable and noninvasive alternative for patients unable to have a hemangioblastoma removed surgically. 

Prognosis

Removed hemangioblastomas in patients without VHL usually have an excellent long-term prognosis without recurrence. However, if a hemangioblastoma has damaged structures or nerves in the spinal cord, brain, or retina, those may not resolve. 

Although there have been technological advances in surgery and treatment options for patients with VHL, the median life expectancy is about 49 years. A lowered life expectancy is primarily due to continued disease progression of new hemangioblastomas and other tumors in the body.

A Word From Verywell

Getting a diagnosis of hemangioblastoma can be frightening. However, there are effective treatments and medical professionals who can help. It is essential to understand your diagnosis and review treatment options with your physician.