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A hemangioblastoma is a benign growth of the central nervous system. These tumors can develop in both the brain and the spinal cord, most often arising in the membrane layer known as the meninges. The most common treatment for a hemangioblastoma is to remove the tumor via surgery. Because these tumors are benign, removal is almost always curative. The exception is in cases where the tumor has an underlying cause such as a genetic defect that predisposes the individual to developing this type of tumor.
Hemangioblastoma tumors are rare, comprising approximately 1 percent to 2.5 percent of tumors that arise in the intracranial region. Most develop in the rear portion of the cranium, with the second most common location for the tumor to develop being the spinal cord. It is rare for the tumor to arise in other locations, but hemangioblastomas occasionally can develop in the optic nerve or peripheral nerves.
Most cases of hemangioblastoma are spontaneously developing single tumors, but people with a heredity disease called von Hippel-Lindau (VHL) disease have a greatly increased risk of this type of tumor. Many people with VHL disease have recurring hemangioblastomas as well as tumors of the kidneys and adrenals. Because these tumors are likely to recur in people with VHL disease, treatment of the tumor is not always successful.
Because most tumors do not recur and typically are removed with relative ease, surgery is the standard treatment for a hemangioblastoma. This treatment is avoided only in cases where the patient’s health is too poor to undergo surgery or in other situations where the risks outweigh the benefits. In some cases, for example, people with VHL do not undergo surgery unless strictly necessary, because of the likelihood of tumor recurrence and an increased risk of complications.
The goal of surgery is always a complete removal of the tumor and the preservation of surrounding brain or neural tissues. In most cases, this is achievable because of the distinctive color of the tumor and the clear demarcation between the tumor and the spinal cord or brain. Hemangioblastomas are a vascular type of tumor, which means they are fed by blood vessels that are part of the tumor itself. Because of this, intraoperative bleeding and severe blood loss is one of the main risks of this surgery. To reduce the risk of serious bleeding, the surgeon must pay careful attention when removing the tumor and coagulating the blood vessels that feed it.