Hemangioblastomas are benign tumors that arise from the linings of blood vessels. Hemangioblastomas can form in the brain, spinal cord, and retina (back of the eye). At The Spine Hospital at The Neurological Institute of New York, we specialize in spinal hemangioblastomas.
Spinal hemangioblastomas are usually located on the surface of the spinal cord. They account for 5-10% of intramedullary tumors, or tumors that arise on or in the spinal cord.
Hemangioblastomas are one kind of hemangioma, or tumors of blood vessels.
Because of their slow-growing and benign nature, small hemangioblastomas that cause no symptoms may not need treatment. These are often found incidentally (in passing) and can simply be followed with annual imaging scans.
Larger hemangioblastomas can cause a variety of symptoms as they compress the spinal cord. Symptoms may include: numbness or pins and needles in the arms or legs, clumsiness of the hands, weakness in the arms or legs, difficulty with gait, and/or compromise of bowel and bladder function. Exact symptoms depend on the tumor’s size and location.
Causes and Risk Factors
Hemangioblastomas arise from primitive blood vessel cells. They are most common in the early and middle decades of life, and affect men and women equally.
In most cases, patients have a single hemangioblastoma. These are called sporadic cases. However, patients with the inherited genetic condition von Hippel-Lindau Disease are predisposed to developing multiple hemangioblastomas, as well as cysts, in all parts of the body, including the kidneys and other organs.
Tests and Diagnosis
Hemangioblastomas are diagnosed by MR (magnetic resonance) scans and/or CT (computed tomography, also known as CAT scans). MR scans use magnets, radio waves, and computer technology to produce images of organs and tissues like the brain and spinal cord. CT scans use a combination of X-rays and computer technology to produce detailed images of bones and soft tissues.
Either scan may be performed after the injection of a contrast-enhancing agent, a substance that enhances visualization of the lesion on the scans.
Spinal angiography, or a study of the blood flow around the tumor, may also be performed as a part of surgical planning. This study is especially useful for hemangiomas; as tumors of blood vessels, they have robust blood supplies.
Hemangioblastomas can often be differentiated from the more commonly occurring intramedullary tumors (astrocytoma, ependymoma) because of their location on the spinal cord surface as well as their more abundant blood supplies.
The treatment of choice for symptomatic spinal cord hemangioblastomas is microsurgery, or removal using a surgical microscope and very fine operating tools. Most tumors can be completely removed using standard microsurgical techniques, and neurological function can usually be preserved (see video).
Microsurgical resection of intramedullary spinal cord hemangioblastoma
Radiation therapy may be useful if the entire tumor cannot be safely removed in surgery.