WHAT IS HEMANGIOBLASTOMA?
A hemangioblastoma is a benign tumor that originates in the central nervous system, developing dominantly in the brain (cerebellum, brain stem) or the spinal cord, and rarely in the retina. The tumor is vascular, meaning it forms in the lining of blood vessels located in the central nervous system.
These tumors are very rare, making up about 3% of spinal tumors, and 2% of all brain tumors. Even though they are slow-growing tumors, are non-cancerous, and do not spread to other parts, they can cause serious neurological problems and complications. However, with their rate of growth, the tumors can be treated before they can cause any problems.
Hemangioblastomas are most likely to occur in:
Hemangioblastomas if not treated on time and left to grow large can lead to complications such as:
- People with the inherited gene mutation causing Von Hippel–Lindau (VHL) syndrome.
- People between the age range of 40 and above.
On a visit to the hospital, your doctor will ask about the symptoms you are experiencing, examine your medical history, and perform a physical examination. Thereafter, several grey tests may be recommended.
- Damage to brain cells
- Nervous system breakdown
- Abnormal high production of red blood cells.
The above-mentioned imagery tests provide images of the tumor and the surrounding swollen tissues.
However, since hemangioblastomas are very rare and occur in similar ways to other tumors, diagnosing them through these tests may be difficult. There’s a more engaging imagery test, which can be used to make an actual diagnosis, and it is called “spinal angiography/angiogram“.
Spinal angiography is used when a diagnosis can not be made through other imagery scans. This test is done by releasing a special dye into a spinal artery through a catheter that has been inserted through a blood vessel in your thigh and up into the spinal artery.
Through the dye, the tumor can be established on a monitoring screen. The tumor’s size, shape, and characteristics can be determined. Hemangioblastomas are known to be vascular, composed of stromal cells which are blood vessels that release a lot of blood. Once this characteristic is identified, a diagnosis is made.
Treatment of hemangioblastomas occurs after a detailed diagnosis of the tumors. Through the diagnosis, your doctor can decide which treatment option is best for you. There are two treatment options for hemangioblastomas. They include:
- Computed Tomography (CT) scan
- Magnetic Resonance Imaging (MRI)
- Ultrasound; to locate the tumor.
This involves the surgical removal or excision of the growth (tumor), making sure the tumor nodule is removed completely. Hemangioblastomas in the cerebellum or brainstem are often removed by retromastoid (suboccipital) craniotomy.
If the removal of the entire tumor is successful, then the condition is cured, but if some regiments of the tumor remain, there is a high tendency of it developing again.
- Conventional (Open) Resection Surgery
In most cases, this noninvasive procedure is preferable to a resection surgery. Here, a concentrated amount of radiation is focused on the tumor to destroy it. This takes just one session to perform, but it may take 6 to 12 months for the tumors to be destroyed and wiped out. It is an outpatient procedure and after the treatment, you can go back to your normal activities.
Radiosurgery is more effective and is a better option for treating hemangioblastomas especially if they are located in parts of the brain or spinal cord that are not easily accessible or easy to excise.
This procedure does not have the complications of a resection surgery such as the risk of bleeding, damage to the nerves, or surrounding blood vessels.
Where Von Hippel-Lindau (VHL) syndrome is the cause of the tumor, the tumor can reoccur or develop in another site of the brain or spine. Therefore, a thorough evaluation should be carried out on you to check for Von Hippel-Lindau disease and if it is discovered, you should be monitored regularly for new tumors.
PROGNOSIS FOR HEMANGIOBLASTOMAS
Research has shown that there is a survival rate of 95% and 91% for 10 and 5 years respectively, and a mortality rate of 9% for 10 years.
Long-term complications are unlikely to develop if hemangioblastomas are treated successfully unless the tumor grew too large and damages nerves, blood vessels, and tissues surrounding it. If such damages occurred, then you should talk to your surgeon about the problems that may arise and how to take care of them.
Once treated for the tumors, make sure to make regular visits to the hospital for monitoring especially if you have VHL as an underlying condition. And if unusual symptoms arise after surgery, make a complaint to your surgeon to know the next step to take.
- Stereotactic Radioactive Surgery (Radiosurgery)
The location of a hemangioblastoma determines the symptoms that are likely to be experienced by the patient.
Cerebellar hemangioblastomas may result to:
- Hydrocephalus–excess cerebrospinal fluid–due to the pressure the tumor may put on the cerebellum.
- Interruption in the supply of the cerebrovascular fluid (CSF) leading to severe headaches and other related symptoms.
- Sensory loss
- Problems in balance and coordination.
Hemangioblastomas that develop in the spinal cord may result to:
- Reduce motor functions
- Weakness especially in the legs and hands.
- Muscular numbness.
- Bladder and bowel incontinence.
Retinal hemangioblastomas are most likely result in:
- Retinal detachment
- Lack of color coordination
- Loss of sight
General symptoms may include:
- Nausea and vomiting
Hemangioblastomas mostly occur on their own, with no definite cause. However, in some cases, there may be certain reasons for their development, or they can be associated with other health conditions.
Causes may include:
- Von Hippel–Lindau (VHL) syndrome; a genetic condition where an affected patient develops benign and malignant tumors in every area of the body.
- Pancreatic cyst.
Is Hemangioblastoma dangerous?
Hemangioblastomas are benign tumors that develop in the brain or spine. They are slow-growing tumors that may take a while to cause complications but when they occur, they are serious causing neurological impairments, visual loss, nervous breakdown, etc.
How fast do Hemangioblastomas grow?
Hemangioblastomas have been tagged as slow-growing tumors. They grow in an inconsistent pattern, with an active growth period of within 13 to 15 months and an inactive growth period of 19 to 25 months.
Is Hemangioblastoma hereditary?
Hemangioblastomas may occur sporadically with no definite cause. However, when it is considered hereditary is when it is associated with Von Hippel-Lindau syndrome, which is a genetic and hereditary disorder.
Is Hemangioblastoma cancer?
No, it is not. Hemangioblastomas are benign (noncancerous) and do not spread to other tissues or organs asides from the ones it affects.
Can VHL be cured?
There is no cure for Von Hippel-Lindau syndrome. The condition leads to the development of tumors in several areas of the body. On early detection of the tumors, they can be treated through surgeries.