Hemangioblastomas (HGB) are benign neoplasms of the vasculature of the central nervous system and retina. HGB occur most commonly in the cerebellum (76%), but also may arise in the cerebral hemispheres, brainstem, spinal cord, or retina. HGB occur slightly more frequently in males than females. Approximately 20-25% of HGB occur as part of von Hippel-Lindau disease, though they also occur sporadically. HGB associated with von Hippel-Lindau disease tend to be multiple, whereas sporadically occurring HGB are usually singular. These tumors may be solid or cystic lesions, which will enhance with contrast on imaging.
HGB may be symptomatic or asymptomatic. Symptoms from lesions in the cerebellum and brainstem are typically caused by the tumor pushing on surrounding structures; these symptoms most commonly include:
These tumors may also cause hydrocephalus, which is an accumulation of cerebrospinal fluid in the brain, and may be life-threatening. HGB of the spinal cord may cause weakness, loss of sensation, imbalance, and difficulty walking, as well as pain. HGB of the retina (part of the eye) are often asymptomatic, but may present with primary visual disturbances and even blindness.
HGB may be evaluated using CT or MRI. Occasionally, an angiogram is performed, especially when surgery is being considered for treatment. Once a hemangioblastoma is discovered, additional scans may be necessary to determine if the patient has von Hippel-Lindau disease.
In cases of sporadic HGB (not associated with von Hippel-Lindau disease), surgical treatment may be curative. However, HGB may be located in inaccessible areas, rendering them dangerous to remove. Because HGB are highly vascular tumors (containing many blood vessels), it is usually helpful to performembolization prior to removal in order to reduce blood loss during surgery, which can make surgery safer and faster.
Radiation may be helpful to reduce tumor size and slow growth. This is typically used for patients that cannot undergo surgery for a variety of reasons including: the patient is a poor surgical candidate, the tumor in an inoperable location, or for multiple small deep lesions.
Julie Kretzer and Justin F. Fraser, MD
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Hussein, M. Central nervous system capillary haemangioblastoma: the pathologist's viewpoint. Int J Exp Path. 2007; 88: 311-24