Hemangioblastoma Center

Overview

The Hemangioblastoma Center at Massachusetts General Hospital provides comprehensive care for patients with both sporadic and familial (hereditary) hemangioblastomas as well as the condition called hemangioblastomatosis. The Hemangioblastoma Center is a collaborative program of the Department of Neurosurgery and the Division of Hematology-Oncology of the Mass General Cancer Center led by Othon Iliopoulos, MD, and Brian Nahed, MD, MSc.

Our goal is to use cutting-edge research and clinical protocols to treat every patient with an individualized treatment plan.

What Is a Hemangioblastoma?

Hemangioblastomas are benign vascular tumors that can form in the linings of blood vessels in the brain, spinal cord, root of the nerves and retina. Brain hemangioblastomas may be hereditary or may occur sporadically without association with known genetic factors.

At the Hemangioblastoma Center, we see patients with von Hippel-Lindau (VHL)-related and non-VHL-related hemangioblastomas and hemangioblastomatosis.

• VHL Disease: VHL is a rare genetic disease that can cause tumors to develop in many areas of the body. The tumors can be benign or cancerous and may cause many serious issues for patients. Patients with VHL disease are at risk of developing hemangioblastomas in the brain, spine, nerve roots and the eye (retina), as well as kidney cancer, pheochromocytoma (tumor of the adrenal glands), neuroendocrine tumors of the pancreas and other lesions.
• Sporadic hemangioblastomas: Sporadic hemangioblastomas are not linked to any hereditary disease. They usually occur in a single location.

Sporadic or VHL-related hemangioblastomas have variable patterns of growth rate. Over time, growing hemangioblastomas can press on the brain and cause headaches, muscle weakness, tingling in the arms or legs, loss of senses and other problems. Hemangioblastomas have also been known to cause a loss of balance and a buildup of spinal fluid in the brain.

Hemangioblastoma Causes

Hemangioblastomas may occur as part of a genetic condition, such as VHL Disease. Up to 25% of all hemangioblastomas are attributed to a hereditary condition and in these cases multiple tumors may form. For the majority of hemangioblastomas there is no known cause.

Hemangioblastomas, which form from blood vessel cells, are most common either early in life or during middle-age. They equally affect men and women. Sporadic hemangioblastomas commonly form only one tumor.

Hemangioblastoma Symptoms

The size and location of a hemangioblastoma will determine the severity of any symptoms. Known as a slow-growing tumor, when small, it is common for a hemangioblastoma to show no symptoms. As the hemangioblastoma grows or if it is in a location that puts pressure on the brain, a mix of symptoms may occur, sometimes starting subtly and growing over time. Potential hemangioblastoma symptoms include:

• Headaches
• Loss of balance or coordination
• Nausea and vomiting
• Muscle weakness
• Numbness or weakness in arms or legs
• Impaired bowel and bladder function

Evaluation and Diagnosis of Hemangioblastoma

We provide comprehensive evaluation and appropriate genetic counseling and testing for VHL disease to identify whether a patient has sporadic or VHL-related hemangioblastoma.

• Hemangioblastomatosis: This is a rare condition where hemangioblastoma cells are growing extensively in a part of the brain known as the leptomeninges and in the spine.
• Retinal Angioma: The term “retinal angioma” has been used to refer to lesions that are most likely retinal hemangioblastomas. Retinal angiomas may indicate the presence of a genetic disease (VHL) or the existence of hemangioblastoma tumors in the brain or the spine. Our center sees and evaluates patients with retinal angiomas.

Hemangioblastoma Treatment

Treatment of hemangioblastoma typically involves surgery, which can often fully remove the tumor. This microsurgery option follows standard techniques, and doctors have a high success rate in retaining your normal functions while removing the tumor, especially in cases of sporadic hemangioblastoma.

In rare cases where surgery is not feasible, stereotactic radiosurgery, a type of hemangioblastoma radiation therapy, can send a concentrated dose of radiation to the hemangioblastoma. The radiation aims to stop blood flow to the tumor, reducing it in size over time.

Hemangioblastoma FAQs

Can Hemangioblastoma Be Cured?

Surgical treatment for hemangioblastomas is highly successful in removing the tumor while preserving a patient's normal function.

Are Hemangioblastomas Malignant?

Growing from the cells of a blood vessel, a hemangioblastoma tumor is benign.

How Common Is Hemangioblastoma?

A hemangioblastoma tumor is not a common tumor. They account for about 3% of all spinal tumors and roughly 2% of all brain tumors.

Are Hemangioblastomas Hereditary?

A hemangioblastoma tumor may be due to a hereditary condition. Up to 25% of all hemangioblastoma cases are linked to genetic diseases, such as VHL.

What to Expect at the Hemangioblastoma Center

Before you arrive at the Mass General Hemangioblastoma Center, your medical records and imaging will be reviewed by your care team. At your appointment, you will first meet with a nurse practitioner and medical geneticist. You will then meet with your physicians, who will work with you to formulate a treatment plan. Depending on your needs, you may have a long-term follow-up by the center.

The decision of when and how to treat hemangioblastoma is made in the Hemangioblastoma Center by multidisciplinary a team of doctors specializing in treatment of this tumor. Your treatment may include:

• Surgery
• Radiation therapy
• Targeted therapy and clinical Trials

A Multidisciplinary Care Team

The Hemangioblastoma Center is a collaborative program of the Department of Neurosurgery and the Division of Hematology-Oncology of the Mass General Cancer Center led by Othon Iliopoulos, MD, and Brian Nahed, MD, MSc, and includes clinicians trained in:

• Medical oncology
• Neuro-oncology
• Neurosurgery
• Radiation oncology
• Genetics
• Neuropathology

The clinic is made up of specialists with experience in treating hemangioblastomas, including:

• Medical oncologists
• Neuro-oncologists
• Neurosurgeons
• Radiation oncologists
• Nurse practitioners
• Geneticists
• Social workers

Other Resources

• Department of Neurosurgery
• Cancer Center
• Center for Cancer Research
• Department of Radiation Oncology
• Mass General Neuroscience

Research

Massachusetts General Hospital researchers work on understanding the etiology of hemangioblastoma tumors and discovering effective treatments for the disease.

Researchers use cutting-edge molecular analysis of cells derived from human hemangioblastoma tumors, hemangioblastoma cell lines established at Mass General and animal models to discover and validate therapeutic targets for the disease.

• Nahed Laboratory
• Shannon Stott Laboratory
• Iliopoulos Laboratory