What is Hemangioblastoma?

 

Hemangioblastoma is a benign tumor of the brainstem or spine that grows slowly. It's a well-defined growth that appears on the inside of your blood vessels' lining. Hemangioblastomas are benign tumors that do no damage to surrounding tissue and can be treated with surgery or targeted radiation.

Overview of Hemangioblastoma

Hemangioblastomas develop in the brain, spinal cord, and retinal blood vessels. These tumors can be detected early and surgically removed before they become too large. The tumors are harmless. This implies they won't turn malignant. They can, however, push on parts of the brain, causing a variety of neurological symptoms.

Causes

Hemangioblastoma is a kind of cancer that develops without apparent reason. As a result, they are hard to forecast. Recurrent hemangioblastomas might be an indication of a hereditary illness called von Hippel-Lindau disease (VHL).

As a result of VHL, a hemangioblastoma develops. It is a hereditary disorder that is passed down through the generations. VHL is responsible for about 20% to 25% of all hemangioblastoma instances. It affects one out of every 36,000 people.


The majority of hemangioblastomas are solitary tumors. Multiple tumors might arise at the same time. However, this is more common in persons who have VHL.

The VHL gene is mutated as a result of VHL. This causes benign tumors to form in a variety of locations throughout the body. The disease is discovered at a young age. VHL has neither a cure nor a treatment. Tumors are surgically removed as the primary therapy.

Complications and risks

Hemangioblastoma develops at random. Those with VHL are the most vulnerable. Because the tumors are benign, they may not require treatment. However, if they become too large, they might begin to cause specific symptoms.‌

In persons with VHL, hemangioblastomas develop repeatedly. For 82 percent of such group, they are the cause of death.

Hemangioblastoma Signs and Symptoms

Tumors that are large exert pressure on your brain and spine. The cerebellum and brainstem are often pressed upon by hemangioblastoma. The following are some of the most common signs and symptoms of one:

  • Headache
  • Coordination problems
  • Imbalance
  • Vomiting
  • Nausea

The cerebellum, located close to the brainstem, is a little region of the brain. The "little brain" is another term for it. The balance, mobility, and coordination of your body are all managed by your cerebellum. Pressure on the cerebellum might make it difficult to move.

The following symptoms may result from pressure on the brainstem and spinal cord:

  • Motor function impairment
  • Deficiency of muscle
  • Your limbs are numb.
  • Gastrointestinal problems
  • Impaired bladder function

Cerebellar edema is a kind of edema that affects the brain and spinal cord Hemangioblastoma can induce swelling in the brain by putting pressure on blood vessels in the brain. When there is too much fluid in your brain, it causes swelling. This is called cerebral edema. It's possible that you'll die as a result of this.

Cystic lesion within the skull. Cerebrospinal fluid can fill a cystic brain lesion. These, like other cancers, push against the brain. Cystic brain lesions, on the other hand, can obstruct the flow of cerebrospinal fluid and cause it to leak into other parts of the brain. If left untreated, this might result in brain damage.

Diagnosis of Hemangioblastoma

A hemangioblastoma can be diagnosed using one of three ways.

  1. A CT scan is a type of imaging that uses a computer to create a three-dimensional image. This approach involves taking a series of X-rays from various angles around your body. Cross-sectional pictures of your bones, blood arteries, and soft tissue are created by a computer processing the images. These X-rays will reveal a hemangioblastoma.
  2. Magnetic resonance imaging (MRI) is a type of imaging that (MRI). This procedure creates comprehensive pictures of your organs and tissues using a magnetic field and radio waves. A functional MRI (fMRI) is a type of MRI that generates pictures of blood flow in specific locations of the brain.
  3. Angiography of the brain. A contrast dye is injected into your circulation in this manner. An angiography shows irregularities in your blood arteries by causing the bloodstream to show up on an X-ray.

Treatment for Hemangioblastoma

The conventional therapy for a hemangioblastoma is surgery. Unless the entire tumor cannot be removed, this usually heals the problem. Some of the diagnostic tools discussed above may be used by your doctor to track your recovery following surgery.


For patients with VHL, surgery may be able to completely eradicate the condition. Only about a quarter of patients who have surgery for a hemangioblastoma require further surgery.

Radiosurgery with stereotactic accuracy (SRS). If surgery is likely to cause issues, this sort of radiation treatment may be employed. It entails administering a high-dose of radiation to the hemangioblastoma.

The radiation beams cut off blood supply to the tumor. Over the course of several months, the tumor shrinks. The procedure's purpose is to stop the tumor from developing.

SRS targets malignancies with precise, concentrated radiation beams. The beams use 3D imaging to provide focused radiation to the afflicted region while causing minimal damage to the surrounding tissue.

A single session of SRS for the brain and spine is generally required. There is little probability of danger or difficulties. SRS has extremely minimal negative side effects. The majority of them are only transitory.