Hemangioblastoma - Symptoms & Causes

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What is a hemangioblastoma?

A hemangioblastoma is a rare, typically benign tumour that arises from the vascular system, most commonly occurring in the brain and spinal cord. These tumours are highly vascular, meaning they contain many blood vessels, and are most often found in the cerebellum, retina, brainstem, or spinal cord. Although hemangioblastomas are benign, they can significantly impact a patient's health.

Hemangioblastoma vs hemangioma

While both hemangioblastomas and hemangiomas involve blood vessels, they are distinctly different:

Hemangioblastomas are rare, benign tumour of the central nervous system, often associated with von Hippel-Lindau disease when multiple lesions are present. They can affect neurological function depending on their location.
Hemangiomas are common, benign tumour of the blood vessels that typically appears on the skin or liver. Hemangiomas are usually superficial and do not typically cause serious problems unless they are located in critical areas affecting organ function.

What are the symptoms of a hemangioblastoma?

The symptoms of a hemangioblastoma depend on the tumour's location but may include:

Headaches
Dizziness or vertigo
Nausea and vomiting
Visual disturbances
Ataxia, or difficulty coordinating movements
Weakness or sensory changes, especially with spinal cord involvement

What causes a hemangioblastoma?

The exact cause of a hemangioblastoma is not entirely understood. However, a significant proportion of cases are associated with von Hippel-Lindau disease, a genetic disorder that leads to multiple tumors forming in the body. Besides hemangioblastomas in the central nervous system, patients with VHL can also develop adrenal gland and kidney tumors. Genetic mutations in the VHL gene are responsible for this condition.

Despite being benign, hemangioblastomas can lead to severe complications, primarily due to their location within the central nervous system:

Hydrocephalus, or accumulation of cerebrospinal fluid, due to blockages caused by the tumour
Neurological deficits, such as motor and sensory disturbances, incoordination of movement or unsteady gait
Hypertension if the tumour affects certain areas of the brain involved in blood pressure regulation

How do you prevent a hemangioblastoma?

There are no known preventative measures specifically for hemangioblastoma. In individuals with von Hippel-Lindau disease, genetic counselling and regular surveillance for new tumors may help manage the condition and prevent complications.