Observation of Hemangioblastomas

Hemangioblastomas are typically benign, vascular tumors that can occur in the brain or spinal cord. Symptoms from a hemangioblastoma will vary depending on the size and location of the tumor.

The most common symptoms a patient may experience are headaches, dizziness, difficulties with balance, and nausea. Hemangioblastomas are often associated with von Hippel-Lindau (VHL) disease, a rare, inherited disorder that leads to tumors and fluid-filled sacs (cysts) in the body, but hemangioblastomas but can also appear sporadically.

While surgery is a common treatment, not all hemangioblastomas require immediate intervention. In some cases, observation—also called "watchful waiting" or "active surveillance"—is appropriate.

The rate at which a hemangioblastoma grows can likewise vary considerably. The term hemangioblastoma refers, in part, to its vascular origin—it arises from cells in the lining of blood vessels.

Some hemangioblastomas may remain stable for years, while others may exhibit gradual or episodic growth. As a result, the decision to treat a hemangioblastoma as well as the recommended treatment typically depends on factors such as the tumor's size, growth rate, and whether it is causing symptoms.

Treatment options for hemangioblastomas include surgery, which aims to remove the tumor completely, and in some cases, radiation therapy, particularly for tumors that are not able to be safely removed by surgery.

When a doctor suggests observation for a hemangioblastoma, it means they want to keep a close eye on the tumor without jumping into treatment right away. Patients are good candidates for observation when a hemangioblastoma is small, not causing any symptoms, and not causing any neurological problems.

This approach is also considered for patients with multiple tumors, as seen in von Hippel-Lindau (VHL) disease, where the risks of repeated surgeries may outweigh the benefits of removing every tumor immediately upon discovery.

The rationale behind this “watchful waiting” strategy is to balance potential risks of treatments such as surgery or radiation therapy against the current impact of the tumor on the patient's health. Since treatments can carry risks and side effects, the decision to intervene is weighed carefully against the potential benefits, especially if a tumor is stable and not causing harm.

Surgery might be deferred for hemangioblastomas located in difficult-to-reach or sensitive areas where surgical risks are especially high.

Additionally, observation may be preferred if a patient has other diseases that make surgery particularly risky to undergo unless absolutely necessary.

When a hemangioblastoma is observed, it is crucial that the patient have a regular monitoring schedule in place with their care team. This approach typically involves MRI scans every 6 to 12 months to assess the tumor's size and growth rate.

The frequency of monitoring may be adjusted based on changes in the tumor's behavior or if new symptoms arise. If the tumor remains stable and symptom-free, observation may continue indefinitely.

However, if there is evidence of growth or new symptoms—such as headaches, neurological deficits, or other signs indicative of increased pressure inside the brain or spinal cord compression—then the treatment plan may shift from observation to active intervention.

The potential need to pivot toward active treatment highlights the importance for patients under observation to maintain regular follow-up appointments and promptly communicate any new symptoms to their healthcare provider. This approach ensures any necessary adjustments can be made in a timely manner.

Observation is often preferred for small hemangioblastomas that are not causing symptoms. The primary risk associated with observation is the potential for the tumor to grow or become symptomatic over time, which could necessitate more urgent and potentially riskier treatment measures. On the other hand, observation avoids the potential complications of more invasive treatments.

Surgical resection and radiation are considered when a hemangioblastoma is symptomatic or shows signs of growth. The goal of surgery is to remove the tumor completely, which can be curative.

However, surgery carries inherent risks. Removing hemangioblastomas can lead to significant bleeding, stroke, and could even be fatal.

Your care team will do everything in their power to ensure the risk is close to zero—but it is not zero.  

The proximity of these tumors to critical brain or spinal cord structures can also increase the risk of neurological damage, resulting in new deficits postoperatively. Other risks include the standard risks of infection and the possibility of tumor recurrence, particularly if complete resection is not achieved.

Radiation offers a non-invasive alternative to surgical intervention, but it is not without its own set of risks. The most serious risk is potential damage to adjacent healthy tissues, which can lead to neurological complications. There is also a small possibility of creating secondary tumors due to radiation exposure. Furthermore, there is the risk that radiation may not fully control the tumor growth, necessitating further treatment.

The management of hemangioblastomas is a complex decision-making process that requires expert and ongoing individualization for each patient. This process involves a careful consideration of the risks and benefits of each treatment option.

Any discussion regarding treatment options should be seen through a lens with a keen focus on a patient's quality of life, preferences, and symptoms. A multidisciplinary team approach ensures that all aspects of care are considered, and ongoing follow-up is essential to adapt the plan as a patient's condition evolves.

• Some hemangioblastomas can remain stable for years, while others may exhibit gradual or episodic growth. Not all require immediate surgical treatment.
• Observation is a strategy reserved for small tumors that lack symptoms, or for those in patients with multiple lesions due to von Hippel-Lindau (VHL) disease.
• Candidates for observation are also those for whom the risks of surgery outweigh the benefits, including those with tumors in sensitive areas or with other significant health conditions.
• Regular monitoring is essential during observation to detect any changes in a tumor's size or behavior. Changes may prompt a need for intervention.
• The decision between observation, surgery, and radiation should be made after careful consideration of the individual patient's circumstances, potential risks, and the tumor's characteristics.

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