Hemangioblastoma is a benign and slow-growing tumor of the vessels with abnormal neoplastic growth of the vascular cells. As the name of the cancer indicates, it is highly vascular. It is not metastatic and can be effectively treated if diagnosed early. However, owing to its complex location, it is vital to address its symptoms promptly and treat it effectively.

Where does a hemangioblastoma usually occur?

Hemangioblastoma is usually associated with the central nervous system. These vascular tumors arise in the central nervous system and cause symptoms. The most common locations where a hemangioblastoma can be present are:

• Cerebellum, which is a part of the brain responsible for coordinated movements of the body.
• Brainstem, which includes the midbrain, medulla oblongata, and pons
• Spinal cord
• Eyes, in the retina part
• Sometimes, hemangioblastoma can also occur outside the central nervous system in some organs like the liver, pancreas, etc.

What are the clinical symptoms of hemangioblastoma?

As hemangioblastoma usually occurs in the central nervous system, the symptoms presented in this situation are CNS-related. Some of the most common symptoms which are reported are:

• Headache and lightheadedness
• Nausea and vomiting
• Numbness in the body parts
• Pain in the eye
• Uncoordinated activities and balance problems
• Loss of appetite
• Problems with the vision
• Behavioural changes and aggression
• Any visible or palpable lump in the specific location

These symptoms must never be taken lightly and need immediate care and attention. Any delay may result in the advancement of the disease and ultimate complications.

What are the causes of hemangioblastoma?

The accurate causes of hemangioblastoma, like any other tumor, are neither known exactly nor can be explained precisely. However, owing to several researches, some causes have been deduced, which are as follows:

• Von-Hippel Lindau (VHL) syndrome: It is a genetic disease that is most commonly associated with hemangioblastomas. It is an autosomal dominant disorder and is the most likely cause of hemangioblastoma. In this condition, there is a mutation in the specific VHL gene that results in the formation of abnormal cells. These abnormal cells proliferate uncontrollably, passing all checkpoints, and as a result, hemangioblastoma originates.
• Exposure to the harmful radiation
• Genetic predisposition
• Mutations in the genes
• Metabolic disorders disrupting the normal cell cycle
• Certain bacterial and viral infections, wildly viral, are capable of incorporating themselves into the normal DNA of the host and causing diseases.
• Neurodegenerative diseases
• Exposure to the state of hypoxia

What are the histopathological features of hemangioblastoma?

The histopathological analysis of the tumor indicates mainly two types of cells. These cells are:

• Stromal cells, which are spindle-shaped and are usually vacuolated.
• The second cells form the rich capillary network of the tumor, which is characteristic of these tumors.

In tumors that have a predominantly cystic nature, there is the presence of large fluid-filled spaces. However, the vascular component of the hemangioblastoma remains the same in cystic tumors.

What are the complications of hemangioblastoma?

As hemangioblastoma usually occurs in susceptible locations of the central nervous system, it can lead to various complications even after the surgery. These complications are:

• Loss of coordinated movements
• Loss of vision
• Hemorrhage owing to the vascular nature of the tumor

How to diagnose a hemangioblastoma?

Like most tumors, hemangioblastoma requires precise equipment and diagnostic tools to halt the progress of the cancer at a very early stage to reduce the chances of mortality. In this case, several diagnostic tests which can be used are:

History and Examination:

A history should be taken regarding disease in any other family member to rule out the genetic causes. The nature and time of appearance of symptoms should also be carefully noted. A detailed examination should be done to check for any lumps, etc.

Genetic testing:

As most of the cases of hemangioblastoma are caused by Von-Hippel Lindau (VHL) syndrome, genetic testing is crucial to highlight the causative factor for hemangioblastoma.

Erythropoietin test:

In many cases of hemangioblastoma, there is an increase in erythropoietin levels, as hemangioblastomas are vascular tumors.

ESR:

ESR is not raised in most hemangioblastoma cases, indicating the absence of any infection in the body and alarming that the symptoms may be related to an underlying pathology.

CT- Scan:

A CT- Scan can be done to diagnose a hemangioblastoma in case of an emergency or unavailability of an MRI.

Magnetic Resonance Imaging (MRI):

To provide a precise and more accurate diagnosis, MRI is always the first choice to visually analyze the tumor and surrounding tissue to prevent complications in surgery. An MRI provides a clear picture of the extent of the disease and its severity.

Angiography:

As hemangioblastoma is a vascular tumor, angiography can also be done if the information from the MRI is not adequate or there is a risk of complications.

Tumor markers:

Several tumor markers can be used, including the markers for the VHL gene mutations, to diagnose and manage the tumor effectively.

What are the treatment options for hemangioblastoma?

As with all tumors, the treatment options for hemangioblastoma include:

Surgery is done to remove the cancer and is practical and possible in most of the cases. The complete elimination of the tumor must be ensured to avoid any chance of relapse. It is the most effective treatment method for hemangioblastoma.

Radiotherapy is done in cases where surgery is not possible or additional treatment is needed first to reduce the size of the tumor.

Cryotherapy is done mainly in the case of hemangioblastoma in the eye. Laser can treat the delicate vessels of the eye and retina.

Supportive treatment like psychological support, pain management, and light exercise should be provided.

What is the survival rate of hemangioblastoma?

Being a benign tumor, the survival rate of a hemangioblastoma after surgery is done is promising. Hemangioblastoma can have a good prognosis depending upon the stage at which it is diagnosed. The 5-year survival rate for a hemangioblastoma after surgery is 90.3%.

Final Note:

Hemangioblastoma, although benign, can lead to severe outcomes if not treated promptly. It is vital to handle any alarming symptoms with great attention to manage the tumor effectively and prevent any fatal results.