Hemangioblastoma

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at October 2, 2016
StartDiseasesHemangioblastoma

Though many people refer to cancer like it is a single medical condition, there are actually many different varieties of cancer that are characterized by different methods and rates of growth. Hemangioblastomas are a specific type of tumor that primarily affects a person's central nervous system.

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Definition & Facts

Hemangioblastomas are tumors that affect the central nervous system, but they originate from cells in the circulatory system instead of coming from nerve cells. Technically, hemangioblastomas are benign tumors, but their close proximity to important nerves can cause issues.

Though hemangioblastomas primarily develop among people in their middle ages, between the age of 30 to 50, it is possible for younger or older people to get a hemangioblastomas.

This type of cancer is composed of endothelial cells and pericyte cells that line and coat the veins and capillaries in a human body. A hemangioblastoma most frequently occurs in either the cerebellum, brainstem, retinas, or spinal cords.

According to the World Health Organization grading system, hemangioblastomas are grade one tumors which means that their tumor cells almost appear normal and grow very slowly. Hemangioblastomas are very rare, making up one one percent of all brain tumors.

Symptoms & Complaints

A hemangioblastoma normally first develops around middle age, and then it grows very very slowly. Sometimes, no symptoms of hemangioblastomas are even present for years. Over time, the symptoms gradually become worse and worse.

Symptoms tend to vary depending on which parts of the body are affected by the tumor. For example, a hemangioblastoma that is extending along the nerves connected to the eyes can cause issues with eyesight.

If the tumor is putting pressure against the cerebellum, a hemangioblastoma can cause difficulty moving, difficulties with balance, vertigo, or poor muscle tone.

Brainstem hemangioblastomas are even more dangerous because they can affect nerves responsible for movement throughout the face and neck. If a hemangioblastoma in the brainstem grows large enough to block the cerebrospinal fluid channels, a patient can experience potentially life-threatening intracranial swelling.

The main danger of hemangioblastomas is that they are composed of blood vessels, so if they rupture they can cause dangerous bleeding in the brain

Causes

Hemangioblastomas are exceedingly rare, but they have a variety of causes. Sometimes, it is possible for a hemangioblastoma to develop spontaneously after something goes wrong during the cell division process. In general, any carcinogen that increases tumor risk can also cause a hemangioblastoma to develop, but they are quite rare. However, hemangioblastomas are frequently associated with other medical conditions. Hemangioblastomas can also be linked to the growth of other tumors such as pheochromocytomas that affect hormone balances in the body.

If a patient has von Hippel-Lindau disease, which is a genetic disorder caused by a mutated tumor suppressor gene, then they are very likely to develop a hemangioblastoma. Since this genetic condition means that a patient's body does not suppress tumors normally, roughly 60 to 80 percent of all patients with von Hippel-Lindau disease get hemangioblastomas.

Diagnosis & Tests

Normally, the main method of diagnosis for hemangioblastomas is magnetic resonance imaging (MRI)s or computed tomography (CT) scans. In these medical imaging tests, the mass will be present somewhere along the central nervous system. On these types of scans, hemangioblastomas typically present as a well-defined area that is less dense than surrounding tissues.

Unlike other types of brain tumors, hemangioblastoma do not metastasize or spread into a lot of surrounding tissue, and they are not accompanied by other smaller clumps of tumors. Instead, they normally have clearly defined edges and only extend into the roots of nearby nerves. Since hemangioblastomas are so highly associated with von Hippel-Lindau disease, a doctor may also recommend that a patient get genetic testing done to see if they are also suffering from this genetic condition.

A detailed family history and physical examination can often provide information about whether the hemangioblastoma is due to a genetic problem or if it just occurred spontaneously. Once the primary diagnosis is complete, more tests will be needed to determine the severity and extent of the tumor.

An angiography, which is a test that introduces a contrast material into the veins before an X-ray is taken, is particularly helpful because it shows how blood flow is affected by the blood vessels that may be present in a hemangioblastoma.

Treatment & Therapy

Depending on the severity, size, and location of the hemangioblastoma, different steps may be taken. Normally, surgery is the best treatment option because once the tumor is removed, there is not really a concern of other cancerous cells spreading throughout the body. Therefore, after surgery, most hemangioblastoma patients do not need to worry about any more appearances of cancer.

A typical surgery to treat a hemangioblastoma starts by severing and stopping the blood vessels that were feeding into the hemangioblastoma before removing the tumor. This surgery can often be done with microsurgical techniques through a small hole in the cranium.

However, if the hemangioblastoma is very large, patients may need two separate procedures. In the first procedure, doctors will block blood flow to the tumor, and then they will open the cranium to actually remove it. If a patient has von Hippel-Lindau disease, the tumor can start to grow again even after surgery was used to treat it, so doctors often recommend that these patients also take chemotherapy to prevent a recurrence.

In some rare cases, surgery is not recommended because the risks to the patients outweigh the benefits. A patient who is frail, elderly, and taking blood thinners could risk hemorrhaging during surgery, so as long as the hemangioblastoma is not causing any immediate problems, it may be better to leave it alone and monitor growth.

Prevention & Prophylaxis

Unfortunately, there is almost no way of preventing hemangioblastomas from developing. If a patient already has a predisposition towards them because the patient has von Hippel-Lindau disease, then they may benefit from frequent monitoring. Regular doctor's visits can help doctors to notice the tumor and take steps to treat it before it becomes truly problematic.