Hemangioblastoma Treatment in Delhi, India

A hemangioblastoma is a benign (non-cancerous) tumor. It originates typically from the lining of the blood vessels, in the spinal cord and brain, and can also grow at the back of the tissue of the retina. Hemangioblastomas can be treated with surgery and radiation, and it does not affect the surrounding healthy tissue. However, hemangioblastomas may affect regions of the brain causing neurological symptoms.

Associated Anatomy of Hemangioblastoma

Spinal cord, brain, and retina.

Alternate Name of Hemangioblastoma

Hemangioma

Hemangioblastoma Causes

The cause of hemangioblastoma is mostly unknown. It is a rare disease. However, most of the cases are related to von Hippel-Lindau (VHL) syndrome. VHL is a genetic disease with the distinguishing feature of abnormal growth of tumors in different parts of the body.

Hemangioblastoma Symptoms

The symptoms depend on the location of the hemangioblastoma. If the tumor is located in the brain, it may cause

• Nausea
• Vomiting
• Headache
• Loss of balance and coordination

If the tumor develops on the spinal cord, it may cause

• Constipation
• Incontinence
• Muscle weakness
• Numbness

The symptoms develop gradually and the size and location of the tumor determine its persistence or disappearance.

Hemangioblastoma Diagnosis and Tests

Your healthcare provider will ask you about your symptoms, health, and family history. They will advise performing a few tests, such as:

Computed tomography (CT) scan

This technique produces cross-sectional images from multiple angles around the body, from which hemangioblastoma can be detected.

Magnetic resonance imaging (MRI)

A variant of the MRI method, called a functional MRI (fMRI), is used to produce images of blood flow in regions of the brain.

Cerebral angiography

A contrast dye is injected into the bloodstream, so that abnormalities in the blood vessels show up in the X-rays.

Hemangioblastomas can often be differentiated from the more commonly occurring intramedullary tumors because of their location on the spinal cord surface as well as their more abundant blood supplies.

Hemangioblastoma Treatment

• Surgery is considered the standard treatment for hemangioblastomas and usually cures this condition.
• Following surgery, individuals who have had a hemangioblastoma should continue to visit their physician regularly for physical examinations and magnetic resonance imaging (MRI) of the brain.
• Stereotactic radiosurgery is an alternative to surgical resection. It is a non-invasive procedure that uses highly focused beams of radiation to target the tumor.

Risk Factors of Hemangioblastoma

• People suffering from von Hippel-Lindau disease (a genetic condition) are likely to develop multiple hemangioblastomas in addition to cysts, in all parts of the body, including the kidneys and other organs.
• Although hemangioblastomas may develop at any age, this type of tumor is typically found in adults around age 40.

Possible Complications of Hemangioblastoma

If hemangioblastoma is successfully treated, long-term complications are unlikely. The tumors are benign and may not need treatment. At times, if the tumor grows too large, they cause certain symptoms. People having VHL usually exhibit repeated growth of hemangioblastomas. It is responsible for 82% of deaths of people belonging to this group.

Other complications of Hemangioblastoma include

Cerebellar edema

Swelling of the brain due to high pressure caused by hemangioblastoma.

Intracranial cystic lesion

Typically the constituent of a cystic brain lesion is cerebrospinal fluid. The fluid exerts pressure against the brain. However, cystic brain lesions can prohibit the flow of cerebrospinal fluid, causing this fluid to go into other areas of the brain. If it is not treated appropriately, it might cause neurological damage.

Prevention of Hemangioblastoma

The complete removal of the tumor can prevent the disease. After the removal of hemangioblastomas, you will need regular follow-ups with your healthcare provider (neurosurgeon). This will help you in the early treatment of hemangioblastomas if they grow back.

Epidemiology of Hemangioblastoma

• This is a condition mostly in young and mature adults, and it has been found that the tumor is primarily intra-axial infratentorial in this category.
• Intracranial tumors affect around 1% to 2.5% and posterior fossa tumors affect close to 10%.
• The second most common location of hemangioblastomas is the spinal cord, the frequency ranges between 2%-3% of primary spinal cord neoplasms and 7%-11% of spinal cord tumors.
• Male to female ratios in adults is 2:1.
• At any age hemangioblastomas may develop, and the peak age of incidence is around 20 to 50 years of age, but earlier in patients with von Hippel-Lindau disease. However, it rarely affects children.
• One-quarter of the patients are associated with von Hippel-Lindau disease.

Expected prognosis of Hemangioblastoma

If successfully treated, complications are rare in the long term. Once the tumor is successfully removed through surgery, the symptoms stop existing. It indicates a successful long-term prognosis without neurological deficiency and recurrence.

However, in patients with VHL (von Hippel-Lindau), even after the removal of hemangioblastomas, new lesions consisting of small multiple hemangioblastomas recur in remote areas and damage brain nerves. Get advice from your doctor on how to manage the disease to minimize its impact on the quality of life.

Natural Progression of Hemangioblastoma

Hemangioblastoma progressing from solid tumor to cyst with a mural nodule has been suggested in the literature. Typically, patients are presented with symptoms due to mass effect, most often from an enlarging peritumoral cyst. This progression may help in making clinical decisions concerning regular follow-ups and the timing of surgical intervention.

Pathophysiology of Hemangioblastoma

Hemangioblastomas grow attached to the pia mater

• Parenchyma of the cerebellum
• Brain stem
• Spinal cord

Pathogenesis of Hemangioblastoma

Although the pathogenesis of the disease is not clear, the VHL-associated hemangioblastomas are thought to be caused by a mutation in the VHL gene. In irregular hemangioblastomas, up to 50% have mutations or deletions in the VHL gene as well.

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