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Hemangioblastoma
What Is It?Hemangioblastoma is a tumor thought to be derived from cells that are precursors to blood vessels and/or blood cells. While they can occur in several places, these brain tumors most frequently occur in the cerebellum, a part of the brain that is important for motor coordination and balance, and the spinal cord. As might be expected from their blood vessel roots, these tumors tend to be very vascular; in other words, they have abundant blood vessels and therefore can have the tendency to bleed.In addition to their vascular nature, hemangioblastoma can be associated with a tumor cyst, a fluid filled cavity that grows alongside the tumor itself. While most of these tumors are low grade, more benign, they can be somewhat aggressive and can occasionally be more malignant. Overall, this type of tumor is rare. They can occur sporadically, without any obvious known cause. However, some hemangioblastoma occur in association with a familial, hereditary disorder called von Hippel-Lindau (VHL) disease. VHL is a genetic disorder which is autosomal dominant. In other words, if a person receives just one of the abnormal genes from either parent, they can develop the disease. von Hippel-Lindau leads primarily to several different types of tumors that develop in numerous tissues and organs. Hemangioblastoma is just one of these tumors which occur frequently in these patients. What Types of Symptoms Are Typical of Hemangioblastoma?While symptoms are variable from patient to patient, because of their typical location in the cerebellum and spinal cord, there are some common presenting symptoms. The symptoms can often be slow to progress but with sudden exacerbations which can be due to bleeding, cyst growth or other injury to surrounding tissues.Tumors in the cerebellum can lead to difficulty with coordination and gait. If the tumor or cyst involves the 4th ventricle, a fluid-filled space in the area of the cerebellum and brain stem, it can lead to hydrocephalus, which is a blockage of the normal flow of this fluid which leads to increased pressure. It can be associated with headaches, nausea and vomiting. Rarely, the tumor can involve the brain stem itself. For tumors that grow in the spinal cord, pain, arm and/or leg weakness and numbness can all occur. In severe cases this can lead to complete paralysis. In patients who have a hemangioblastoma due to von Hippel-Lindau disease, patients may have symptoms due to tumors in other organs as well as polycythemia. How Is The Diagnosis Typically Made?Like most brain tumors, after a thorough neurological examination, an imaging study such as an MRI scan is usually performed. These studies can identify the tumor, its size and location. There are certain features of these tumors, such as their location and the presence of large blood vessels or hemorrhage, which make diagnosis by MRI possible. However, like most tumors, definitive diagnosis relies on obtaining tumor tissue for evaluation by a pathologist.What Are Some Common Treatments?Patients who present with hydrocephalus will often require a procedure to treat this condition, such as a ventriculoperitoneal shunt. For the tumor itself, surgery is often a primary part of the treatment plan. The goal of surgery is generally to remove the entire tumor. Because these are generally benign tumors, a complete removal is one of the best chances for a cure.Because of the vascularity of hemangioblastoma, some tumors are embolized prior to surgery. This means that an radiologist injects glue or other materials into the large blood vessels to attempt to cut off blood flow. This can sometimes make surgery easier and safer by preventing some bleeding at the time of subsequent surgery. Radiation therapy is another option for the tumor, particularly for residual tumor or an unresectable tumor. The specifics of treatment is highly individualized depending on specific patient and tumor characteristics and therefore cannot be generalized. Each patient must discuss the specific treatment plan with their own physicians. Return to the Nervous System Diseases home page. Important Note: This site is not intended to offer medical advice. Every patient is different, and only your personal physician can help to counsel you about what is best for your situation. What we offer is general reference information about various disorders and treatments for your education. |
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