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Hemangioblastomas are benign growths of the central nervous system, most commonly found in the meninges of the brain or spinal cord. Surgery is the standard treatment, but those with von Hippel-Lindau disease may have recurring tumors. The goal of surgery is complete removal while preserving surrounding tissue, but there is a risk of severe bleeding due to the tumor’s blood vessels.
A hemangioblastoma is a benign growth of the central nervous system. These tumors can develop in both the brain and spinal cord, most often arising in the layer of membrane known as the meninges. The most common treatment for a hemangioblastoma is to remove the tumor through surgery. Because these tumors are benign, removal is almost always curative. The exception is in cases where the cancer has an underlying cause such as a genetic defect that predisposes the individual to developing this type of cancer.
Hemangioblastoma tumors are rare and comprise approximately 1-2.5% of tumors arising in the intracranial region. Most develop in the back of the skull, with the second most common location for cancer to develop is the spinal cord. The tumor rarely occurs elsewhere, but hemangioblastomas can occasionally develop in the optic nerve or peripheral nerves.
Most cases of hemangioblastoma develop single tumors on their own, but people with an inherited disorder called von Hippel-Lindau disease (VHL) have a greatly increased risk of this type of cancer. Many people with VHL disease have recurring hemangioblastomas as well as tumors of the kidney and adrenal glands. Because these tumors are likely to come back in people with VHL disease, treating the tumor is not always successful.
Because most tumors don’t come back and are usually removed relatively easily, surgery is the standard treatment for a hemangioblastoma. This treatment is avoided only in cases where the patient’s health is too precarious to undergo surgery or in other situations where the risks outweigh the benefits. In some cases, for example, people with VHL do not have surgery unless absolutely necessary, due to the likelihood of the tumor coming back and an increased risk of complications.
The goal of surgery is always complete removal of the tumor and preservation of the brain or surrounding neural tissue. In most cases, this is possible due to the distinctive color of the tumor and the clear demarcation between the tumor and the spinal cord or brain. Hemangioblastomas are a type of vascular tumor, which means they are fed by blood vessels that are part of the tumor itself. For this reason, intraoperative bleeding and severe blood loss are one of the main risks of this surgery. To reduce the risk of severe bleeding, the surgeon must be very careful when removing the tumor and clotting the blood vessels that feed it.
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