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Hemangioblastomas

A hemangioblastoma is a benign, highly vascular tumor that can occur in the brain and spine. As it enlarges, it will press on the brain and can cause neurologic symptoms, such as headaches, weakness, sensory loss, balance and coordination problems, and/or hydrocephalus (a build up of spinal fluid). In rare cases, the tumor is discovered as an incidental finding during an evaluation for unrelated symptoms or another disorder. Nearly all brain lesions occur within the inferior back portion of the brain, known as the cerebellum. This part of the brain is especially important for balance and coordination.

Most hemangioblastomas occur as a single lesion. However, some patients develop hemangioblastomas as part of a genetic syndrome called von Hippel Lindau disease (VHL), a genetic disorder inherited from either parent (dominant inheritance). Over their lifetimes, such patients develop multiple tumors within the brain and spinal cord.

Hemangioblastomas occur in two basic forms:

  • Solid tumors consist entirely of tumor cells.
  • Cystic hemangioblastomas are composed of a generally small solid component, adjacent to an oftentimes much larger cystic portion of tumor.

Diagnosis

Hemangioblastomas can be diagnosed by either contrast CT or MRI scans of the brain or spine. Because of its characteristically profuse blood supply, a hemangioblastoma sometimes can be diagnosed with the aid of a cerebral angiogram.

When a family history of von Hippel Lindau is present, there is a strong likelihood that other directly descended family members will be afflicted by this disorder.

Treatment

There are two basic treatment options for hemangioblastoma, surgery and radiosurgery.

Surgery

Surgical resection involves operating on the brain or spine to physically remove a tumor. If a hemangioblastoma can be completely removed and is not associated with von Hippel Lindau disease, then a patient is cured. If the entire hemangioblastoma cannot be resected, there remains a high likelihood that either the tumor will re-grow or that additional cysts may form.

In the typical case of a cystic hemangioblastoma, only the solid portion of the tumor needs to be removed; the adjacent cyst that is drained during surgery, will eventually disappear once the tumor nodule is removed.

Any operation on the brain or spine is invasive and can be associated with risks, such as stroke, infection, anesthetic complication, or neurologic deficits. However, most hemangioblastomas can be safely removed with present day neurosurgical instrumentation.

Radiosurgery

As an alternative to an open operation, stereotactic radiosurgery can be used to target and destroy a hemangioblastoma. A single radiosurgery session will result in the gradual death and eventual shrinkage of a treated lesion. In the case of cystic hemangioblastoma, only the solid portion of the tumor need be ablated with radiosurgery; cyst fluid gradually ceases to be produced and eventually the cyst decreases in size.

The principle advantage of stereotactic radiosurgery is that it is noninvasive and does not carry the risks of conventional brain or spine surgery. Furthermore, some hemangioblastomas are located in areas of the brain that are difficult to approach safely with conventional surgery. In such cases, radiosurgery may be a much safer option for the patient. Moreover, radiosurgery is an outpatient procedure, and does not require any recovery period before a patient can resume a normal lifestyle.

In patients with von Hippel Lindau disease, multiple hemangioblastomas tend to develop over time within both the brain and spine, often necessitating multiple operations over a patient's lifetime. Even when successful, these operations generally take their toll on the patient, often resulting in gradual disability. For these patients, stereotactic radiosurgery can preventing the need for numerous conventional operations.

There are two main disadvantages to ablating hemangioblastomas with radiosurgery:

  • First, radiosurgery typically takes from six months to a year to destroy a hemangioblastoma. Therefore, if a tumor is large and causing significant symptoms from pressure on the normal brain, standard surgical resection is usually necessary. Even after aggressive radiosurgery for cystic hemangioblastoma, fluid continues to be produced within the cyst for several months after treatment. Also, if the cyst becomes large, it too can cause symptoms. Such large cystic tumors can be symptomatic and require surgical drainage, even though the tumor is dying from radiation.
  • Secondly, radiation may cause injury to the normal brain immediately adjacent to the treated hemangioblastoma (see complications of radiosurgery below).

The main potential complication of radiosurgery is the chance that radiation may destroy the tumor but injure the adjacent normal brain. Such damage is called radiation edema, or in severe cases, radiation necrosis. Particularly for small tumors, the likelihood of radiation injury to the brain is low.

The rate of complications is generally much lower than the risk of leaving the tumor untreated or undergoing an open surgical resection. The symptoms produced by radiation edema will often improve significantly with oral steroids.

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