Treatment of Hemangioblastoma

Hemangioblastomas are rare, benign tumors that grow from abnormal blood vessel growth in the brain or spinal cord. Hemangioblastomas are typically associated with von Hippel-Lindau (VHL) disease, which can influence the treatment strategy.

While they are not cancerous, they can cause significant neurological symptoms and complications due to their location, presenting unique challenges during treatment. The right treatment approach for hemangioblastomas depends on various factors, including the tumor size, location, symptoms, and your individual characteristics.

We’ll explore the different treatment options available for hemangioblastoma, so that you and your loved ones can make informed decisions.

For many people with hemangioblastomas, surgery is the primary form of treatment. The goal of surgery is to remove as much of the tumor as possible while protecting your brain and spinal cord in order to relieve symptoms and prevent future complications.

Here is what you can expect during hemangioblastoma surgery: 

Before surgery, you will meet with your healthcare team, including your neurosurgeon, anesthesiologist, and nurses. They will review your medical history, examine you, and answer any questions you may have. This is a great time to share any concerns or thoughts you have about the procedure.

You will be admitted to the hospital on the day of your surgery. After arriving, you will be taken to your room, where your team will help you get ready.

When it’s time for surgery, you will be taken to the operating room, where the anesthesia team will give you medication to make sure you are comfortably asleep and won’t feel anything during the surgery. The anesthesiologist will monitor you carefully to ensure your safety and comfort.

Once you are under anesthesia, the neurosurgeon will begin the surgery. The specific approach and technique used will depend on the size, location, and characteristics of the hemangioblastoma.

The most common approach is microsurgical resection, where your neurosurgeon will use advanced tools and a microscope to carefully locate and remove the tumor. The goal is to remove as much of the tumor as possible while protecting your healthy brain and spinal cord tissue.

 When the surgery is complete, the incision will be closed, and you will be taken to the recovery room. You will slowly wake up from anesthesia, and the nursing team will monitor you closely.

After surgery, your healthcare team will closely monitor you to ensure that you are recovering well. Your team will help to manage any pain or other symptoms you may have, as well as monitor your vital signs and neurological function.

Your healthcare team will give you specific instructions for taking care of yourself after surgery. Physical and occupational therapy may be recommended to help regain your strength and function.

Your healthcare team will guide you on when you can resume normal activities and arrange follow-up appointments for monitoring your progress.

Once you are ready to leave the hospital, your healthcare team will provide detailed instructions about wound care, activity limitations, and any medications you’ll need. You will also schedule a follow-up appointment with your neurosurgeon to monitor your recovery and discuss next steps.

Surgery for hemangioblastoma, while often effective in removing the tumor and alleviating symptoms, carries certain risks. These risks can vary depending on factors such as the location and size of the tumor, the surgical approach used, and your individual health.

Your healthcare team will help you to understand these risks and take every precaution to minimize complications, as well as help you manage any issues that may arise. Some of these risks include:

Surgery in or around the brain and spinal cord has a risk of causing neurological issues, such as muscle weakness, issues with your senses, balance and movement difficulties, or changes in speech or brain function. These changes may be temporary or permanent. If these issues occur, your healthcare team will offer treatments and strategies to help you recover.

Hemangioblastomas involve blood vessels, so there is a chance of bleeding complications during surgery. Intraoperative hemorrhage, or excessive bleeding during surgery, can occur during tumor removal.

Your surgeon is prepared for this and will take steps to stop the bleeding if necessary.

Surgery near the brain or spinal cord may disrupt their protective covering, leading to a fluid leak. This can result in headaches, nausea, vomiting, or meningitis if left untreated.

Surgical repair or placement of a temporary drain can help to fix the leak.

All surgical procedures carry the risk of postoperative infections. In surgery for hemangioblastomas, there is a risk of surgical site infections, meningitis, or brain abscesses.

Your care team will monitor you closely in order to promptly recognize any signs of infection. In the case of an infection, your team will treat you with antibiotics.

Surgery to remove hemangioblastomas may damage surrounding brain or spinal cord tissue, leading to neurological issues or difficulties with functioning. The risk of injury is higher in tumors located in areas of the nervous system that are important for your function.

In some cases, hemangioblastomas may block the flow of cerebrospinal fluid (CSF), or fluid in your nervous system, leading to hydrocephalus, or fluid buildup in the brain. Surgical removal of the tumor may help reduce the obstruction, but hydrocephalus may develop as a complication of surgery, requiring additional treatments.

Even if the tumor is removed, there is a small chance that hemangioblastomas may grow back. Recurrent tumors may require additional treatments.

Anesthesia-related complications are rare, but can occur in patients with underlying medical conditions.

While everything is done to mitigate these risks, the risk is not zero, and complications of the surgery can be fatal.

It's important to remember that while these complications are possible, most patients undergoing surgery for hemangioblastoma have successful outcomes.

Your healthcare team is experienced in managing these risks and will collaborate with you to minimize risks, optimize outcomes, and manage any complications that may arise.

Radiation therapy is another treatment option that can be used for hemangioblastomas. This non-invasive method works by damaging the DNA of tumor cells, preventing them from multiplying. Radiation beams are delivered to the tumor site using specialized equipment, such as linear accelerators or gamma knife radiosurgery systems.

These beams precisely target the tumor, allowing for high doses of radiation to be delivered while protecting your adjacent healthy tissue. It is typically used as an adjunct to surgery or when surgery is not possible.

There are different techniques used in radiation therapy for hemangioblastomas, including:

• External Beam Radiation Therapy (EBRT): EBRT delivers radiation from outside the body using a machine called a linear accelerator (LINAC). Multiple beams are aimed at the tumor from different angles, precisely targeting the tumor and avoiding healthy tissue.
• Stereotactic Radiosurgery (SRS): SRS is a precise, high-does radiation treatmeant used for small to moderate-sized hemangioblastomas located in important areas of the brain where surgery carries higher risks.
• Fractionated Radiation Therapy: Fractionated radiation therapy splits the total radiation dose into multiple treatment sessions (fractions) to minimize damage to healthy tissue. This approach allows for better tolerance of radiation.

The decision to undergo radiotherapy depends on several factors, including the size, location, and characteristics of the tumor, as well as your overall health status and treatment goals. The management of hemangioblastomas may involve a combination of therapies.

Radiotherapy may also be reserved for specific situations where it is deemed necessary or beneficial. Radiation therapy may be recommended for hemangioblastomas in the setting of residual or recurrent tumors, inoperable tumors, or for tumor symptom relief and control.

Radiation therapy for hemangioblastomas is generally safe and well-tolerated, but it does carry some risks. You should be aware of the potential for these complications:

Radiation therapy may lead to tissue damage and inflammation in the surrounding brain or spinal cord, resulting in radiation necrosis, or death of healthy tissue. Symptoms of radiation necrosis may include headaches, seizures, cognitive changes, or focal neurological deficits.

Radiation therapy can cause edema (swelling with fluid) in the surrounding brain and spinal cord tissue, leading to increased intracranial pressure and neurological symptoms. Corticosteroids are medications commonly used to help reduce edema and alleviate symptoms.

Prolonged exposure to radiation may increase the risk of long-term radiation toxicity, including cognitive decline, endocrine dysfunction, or secondary malignancies. It is important to stay up to date with appointments, even long after radiation therapy, so that your healthcare team can detect and manage late-onset complications.

Observation, also known as “watchful waiting,” or active surveillance, is a common approach in the care of hemangioblastomas. This involves regularly monitoring the tumor over time through clinical evaluations and imaging studies without starting treatment right away.

The decision to observe a hemangioblastoma is based on several factors:

Small, asymptomatic hemangioblastomas with stable or slow growth may not pose an immediate threat to your health or quality of life, and the risks associated with treatment may outweigh the potential benefits

You may prefer to avoid surgery or radiation, particularly if the tumor is not causing significant symptoms or impairing daily functioning. Observation allows you to avoid the potential risks of surgery or radiation therapy while still receiving regular monitoring and medical support.

Patients with certain conditions or contraindications to surgery or radiation therapy may benefit from an observation strategy.

Hemangioblastomas often grow slowly, with some tumors remaining stable or even shrinking over time. Observation allows your healthcare team to assess the tumor's behavior and response to treatment or natural history before intervening.

During the observation period, you will have regular follow-up appointments with your healthcare team, typically every 6 to 12 months. The frequency and duration of observation may vary depending on your individual situation and the tumor’s characteristics.

The goal of observation is to closely monitor the tumor while avoiding unnecessary treatment. Tumors may show signs of growth or begin to cause symptoms during the observation period.

If your tumor progresses, your care team may discuss treatment options. If the tumor remains stable or shows evidence of regression without causing significant clinical impact, you may benefit more from continued observation.

• Surgery plays an important role in the management of hemangioblastoma.
• Radiation therapy can be used in certain situations, especially when surgery is not possible.
• Both surgery and radiation therapy carry inherent risk, and treatment options should be weighed after thorough discussion with your care team. 
• Observation can be a good management strategy when the risks of active treatment outweigh the benefits.

• National Institutes of Health (NIH): Hemangioblastoma
• Cancer Research UK: Hemangioblastoma
• American Brain Tumor Association (ABTA): Hemangioma
• VHL Alliance