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A hemangioblastoma is a rare, benign (noncancerous) tumor that develops within the central nervous system (CNS), typically in the brain, spinal cord, or retina. As they grow, these tumors can press on nearby nerves and tissues, causing neurological symptoms.
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A hemangioblastoma is a rare, benign (noncancerous) tumor that develops within the central nervous system (CNS), typically in the brain, spinal cord, or retina. These tumors arise from abnormal clusters of blood vessels and can lead to significant complications depending on their size and location.
Most hemangioblastomas occur as a single tumor, but in some cases, they develop as part of a genetic disorder called von Hippel-Lindau (VHL) disease. People with VHL disease may develop multiple tumors throughout their lifetime in different organs, including the brain, spinal cord, kidneys, and pancreas.
Hemangioblastomas are most commonly found in the posterior fossa (which includes the brain stem and cerebellum). They can also occur in the cerebral hemispheres, spinal cord, or retina.
As they grow, these tumors can press on nearby nerves and tissues, causing neurological symptoms.
Spinal hemangiomas are common, slow-growing, and often asymptomatic vascular malformations found in the spine or brain.
Hemangioblastomas are a type of hemangioma that develop primarily in the brain, spinal cord, or retina, often causing neurological symptoms due to rapid growth.
In most cases, the cause of hemangioblastomas is unknown. However, in individuals with VHL disease, mutations in the VHL gene cause uncontrolled growth of blood vessels, leading to tumor formation.
Your risk of developing a hemangioblastoma may be higher if you have:
If your doctor suspects VHL disease, they may recommend genetic testing to check for mutations in the VHL gene that could affect future generations.
As hemangioblastomas grow, they can put pressure on the brain or spinal cord, leading to neurological complications such as:
Hemangioblastomas are rare. They account for 2 percent of all primary brain tumors and only 3 percent of all spinal tumors.
Approximately 10 percent of hemangioblastoma cases occur in people with VHL disease, which can also lead to tumors in the kidneys, liver, and pancreas.
Hemangioblastomas can develop at any age but are most commonly diagnosed around age 40.
Symptoms depend on the tumor's location and may include:
If your symptoms interfere with daily activities or cause significant discomfort, seek medical evaluation. Early diagnosis and treatment can prevent complications.
Diagnosing a hemangioblastoma can be challenging due to its rarity and similarity to other tumors. Your doctor may use imaging tests such as:
The two primary treatment options for hemangioblastoma are surgical removal and stereotactic radiosurgery (SRS).
Surgery is the gold standard for treating hemangioblastomas, especially when they:
The goal of surgery is to remove the entire tumor, which is successful in 80-90% of cases.
If the tumor is completely removed and is not associated with VHL disease, you may be considered cured. However, incomplete tumor removal may lead to tumor regrowth.
SRS is a noninvasive treatment that uses highly focused radiation beams to target and destroy tumors, without the need for open surgery. It is especially useful for tumors located in difficult-to-reach areas of the brain.
SRS is an outpatient procedure that delivers precise radiation to the tumor while sparing healthy tissue. Most people can resume normal activities the same day as their SRS procedure, but it can take six months to a year for the tumor to shrink or be destroyed.
If hemangioblastoma is associated with VHL disease, additional monitoring and treatments may be required to manage multiple tumors.
Your treatment plan will depend on:
Surgery offers immediate tumor removal, but SRS provides a less-invasive alternative with a lower risk of complications. Your doctor will recommend the best approach for your individual case.
UPMC is nationally recognized for excellence in neurosurgical care. We offer comprehensive care from diagnosis through treatment and beyond. Our team offers the most advanced nonsurgical and surgical treatments for a full range of brain and spine disorders and is engaged in pioneering research to develop novel treatment options.
By UPMC Editorial Staff. Last reviewed on 2025-05-16.
