Causes and Risk Factors of Hemangioblastoma

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Most hemangioblastomas occur for no known reason and are discovered only after symptoms develop. Fortunately, these benign tumors can usually be cured with surgery.

In some cases, hemangioblastomas grow as a result of a genetic condition called von Hippel-Lindau (VHL) syndrome. For tumors associated with VHL, the main risk factor is family history, but research is ongoing to determine the role of environmental and lifestyle risk factors.

In this article, you'll learn more about genetic risk factors and other possible causes of hemangioblastomas.

What is a Hemangioblastoma? - Illustration by Jessica Olah

Verywell / Jessica Olah

Common Causes

The cause of most hemangioblastomas is a mystery. Hemangioblastomas are rare, accounting for just 2% of all brain tumors and 2% to 10% of spinal cord tumors. In most cases, these tumors develop at random.

However, about 25% of all hemangioblastoma cases may develop as part of a genetic syndrome called von Hippel-Lindau (VHL) syndrome. VHL is an inherited syndrome characterized by the growth of a variety of benign and potentially malignant tumors. Most of the time it can be assumed that single tumors are sporadic. Multiple tumors are almost always associated with VHL.

Genetics

Hemangioblastomas occur 1.5 to 2 times more frequently in men than in women. No ethnic predominance has been reported.

Nearly 25% of people with hemangioblastomas develop the tumors as a result of VHL syndrome, a genetic defect that causes capillary growth to go out of control. People with this condition tend to develop benign or cancerous tumors in different parts of the body, including hemangioblastomas throughout the body, such as in the:

  • Brain
  • Spinal cord
  • Retina
  • Inner ear
  • Kidney
  • Adrenal gland
  • Pancreas

The main risk factor for VHL is family history, although mutations to the VHL gene can happen in those without parents who have VHL. If your doctor has high suspicion for VHL, they may suggest genetic testing to help uncover a potential mutation in the VHL gene. 

Lifestyle Risk Factors

Most of the time hemangioblastomas happen sporadically, or for unknown reasons, but in cases of VHL, family history is the main risk factor.

The role that the environment and/or lifestyle plays, if any, in their development and growth is still being researched. However, adopting cancer-preventive behaviors (such as eating a nutrient-dense diet and exercising regularly) is recommended to promote optimal health, especially in those with VHL.

Since VHL is also associated with malignant tumors, those who are diagnosed with this syndrome are encouraged to quit smoking, maintain a healthy weight, exercise routinely, and limit alcohol to decrease their cancer risk. 

A Word From Verywell

Hemangioblastoma is a rare and mysterious condition that can be hard to detect and even harder to treat. If you are feeling symptoms of weakness, vision changes, loss of bladder control, or difficulty with walking, you should see your primary care physician or a healthcare provider immediately. They can connect you with the specialists needed to make an accurate diagnosis.

If a hemangioblastoma is suspected, a team consisting of an oncologist, a neurologist, a neurosurgeon, and a physical therapist will help to explain your treatment options and an aftercare plan that you may expect moving forward.

6 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. UCLA Health. Hemangioblastomas.

  2. Yin X, Duan H, Yi Z, Li C, Lu R, Li L. Incidence, prognostic factors and survival for hemangioblastoma of the central nervous system: analysis based on the surveillance, epidemiology, and end results databaseFront Oncol. 2020;10:570103. doi:10.3389/fonc.2020.570103

  3. National Center for Advancing Translational Sciences: Genetic and Rare Diseases Information Center. Hemangioblastoma.

  4. Taïeb D, Barlier A, Yang C, et al. Somatic gain-of-function HIF2A mutations in sporadic central nervous system hemangioblastomasJ Neurooncol. 2016;126(3):473-481. doi:10.1007/s11060-015-1983-y

  5. Pan, James BS; Sussman, Eric MD; Tayag, Armine MSN; Thompson, Patricia MSN; Chang, Steven D. MD Central nervous system hemangioblastomas. Contemporary Neurosurgery. 2015; 37(25): 1-5. doi:10.1097/01.CNE.0000483965.33921.3c

  6. American Cancer Society. Risk Factors for Brain and Spinal Cord Tumors.

By Shamard Charles, MD, MPH
Shamard Charles, MD, MPH is a public health physician and journalist. He has held positions with major news networks like NBC reporting on health policy, public health initiatives, diversity in medicine, and new developments in health care research and medical treatments.