Living with Hemangioblastoma

Living with hemangioblastoma involves managing a benign, or non-cancerous, but potentially problematic tumor that can occur sporadically or as part of von Hippel-Lindau (VHL) syndrome. These tumors are highly vascular—originating from growth within blood vessels—and can arise in the brain, spinal cord, or retina.

They may cause symptoms, such as headaches, neurological deficits, or hydrocephalus (a buildup of cerebrospinal fluid in the brain), depending on their location. Hemangioblastomas generally grow slowly, which may afford a patient options besides immediate surgical treatment, depending on different factors.

The life expectancy for someone with hemangioblastoma can vary widely based on several factors, including the location and number of tumors, whether the tumors are associated with VHL, a patient's overall health and symptoms, and treatment effectiveness.

There are various prognostic factors associated with longer patient survival:

• Younger age at diagnosis
• Female sex
• Absence of neurological symptoms
• Single tumor at diagnosis rather than multiple tumors
• Higher Karnofsky performance status score, a scale that assesses a patient’s ability to function in various tasks

Life expectancy for patients with VHL, while lower than the general population, has improved with better surveillance and management of the disease. VHL is a genetic condition that predisposes individuals to multiple tumors, including but not limited to hemangioblastomas in the brain and spinal cord.

Life expectancy for VHL patients is not solely determined by hemangioblastomas, but also by the management of other tumors associated with VHL. VHL is also associated with renal cell carcinoma, a form of cancer in the kidneys, and pheochromocytoma, a tumor arising near the kidney that can have serious cardiovascular consequences. 

Surgery is often the first-line treatment for accessible hemangioblastomas, especially if they are symptomatic. The goal of surgery is to completely remove the tumor, which, as mentioned, can potentially be curative for solitary lesions. The success of surgery depends on the tumor's location and the ability to safely resect it.

Stereotactic radiosurgery, or SRS, is a non-invasive radiation therapy technique that delivers a high dose of radiation precisely to the tumor while sparing surrounding healthy tissue. It is an option for smaller tumors or for patients who are not good candidates for surgery.

When hemangioblastomas are small and asymptomatic, especially in patients with VHL disease, observation with regular follow-up may be recommended. This approach is taken to avoid unnecessary interventions while closely monitoring for tumor growth or the development of symptoms.

Life after a hemangioblastoma diagnosis can be marked by a period of adjustment and ongoing medical surveillance. While hemangioblastomas are typically benign tumors, they can still cause significant health issues.

Regular medical appointments are used to monitor for tumor recurrence or progression. It is important for patients to follow closely with their care team to manage any potential long-term side effects of treatment.

Many patients can return to their normal activities, but some may need to make adjustments based on physical and cognitive capabilities. Emotional and psychological support is also important, as the diagnosis itself can be a source of stress and anxiety.

For sporadic hemangioblastomas, recurrence rate is generally lower. However, there is still a risk of recurrence, particularly if the initial surgery did not remove the entire tumor or if there were multiple lesions present at diagnosis.

Patients with VHL have a higher likelihood of developing new hemangioblastomas. These patients may require a more intensive follow-up regimen, including frequent imaging and possibly multiple treatments due to new tumors or tumor recurrence. Due to the risk of recurrence, patients with hemangioblastoma typically undergo regular follow-up imaging, such as MRI, to detect any new growth early.

Without treatment, the symptoms caused by a hemangioblastoma are likely to progress. These symptoms may include headaches, nausea, vomiting (often due to increased intracranial pressure), visual disturbances, balance and coordination problems, and other neurological deficits specific to the tumor's location.

In severe cases, untreated hemangioblastomas can be life-threatening. For example, a large tumor in the brainstem or cerebellum can cause brain herniation, a serious condition where brain tissue is displaced due to increased pressure within the skull. This complication requires urgent intervention.

Supporting a family member with hemangioblastoma involves both emotional and practical help, such as assisting or arranging for help with daily tasks after treatment. By reading this article, you are also educating yourself on the condition—an important way of showing your support.  

Support groups for patients and families can also be comforting while providing practical advice from those who have been in your shoes. Taking care of your own health and seeking ways to share this journey are crucial to supporting life with and after hemangioblastoma.

• Life expectancy for hemangioblastoma patients varies based on factors like tumor location, number of tumors, VHL syndrome status, overall health, and treatment effectiveness.
• Better survival is linked to younger age, female sex, no neurological symptoms, a single tumor, and a higher Karnofsky performance score.
• Regular imaging and side effect management are essential, with follow-up frequency personalized to each patient.
• Many return to normal activities, but some need lifestyle changes and emotional support due to new limitations and diagnosis-related stress.
• Recurrence risk is lower with complete resection of sporadic tumors, while VHL syndrome patients have a higher chance of new tumors developing.

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