Hemangioblastoma - Brain & Spinal Cord 

Introduction
Hemangioblastomas are noncancerous tumors that develop in the brain or spinal cord.  Some hemangioblastomas are caused by an inherited disorder; others occur sporadically.  The tumors may cause a host of neurological symptoms, depending on their size and location.  In most cases, surgical removal cures the condition.

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Anatomy
Your brain is located inside of your skull.  It is the control center of your body.  Your brain controls the way you think, behave, feel, and move your body.  Your brain communicates with the nerves in your body for functions you can control, such as talking or moving your arms and legs.  Your brain also controls the life-sustaining functions that happen automatically, including your heartbeat, body temperature, blood pressure, and body metabolism.
 
The spinal cord is a column of nerves that extends from your brain.  The spinal cord is located inside of your spine.  Your spinal cord and brain exchange nerve messages about muscle movements and sensations that are felt on your skin. 
 
The cells in your brain and spinal cord need oxygen to function and survive.  Arteries and veins carry oxygen in blood throughout your body and central nervous system in an ongoing process called circulation.  Capillaries are tiny blood vessels that connect your arteries and veins.  They help to deliver nutrients to cells and carry waste products away.

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Causes
Hemangioblastomas are noncancerous masses of abnormal blood vessels and cells that develop in the brain, and less frequently in the spinal cord.  Hemangioblastomas are composed of tumor cells that are surrounded by an abnormal capillary network.  Most hemangioblastomas occur sporadically, but some may be caused by an inherited genetic disorder, von Hippel-Lindau disease.

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Symptoms
Symptoms of hemangioblastomas depend on the size and location of the tumor.  Although hemangioblastomas are not cancerous, their presence can cause nervous tissue injury or damage.  People may experience a progression of mild neurological symptoms followed by a sudden increase in symptoms.  Hemangioblastomas most frequently develop in the cerebellum, brain stem, and spinal cord.
 
The cerebellum is the part of the brain that controls balance and coordinated movements.  People with tumors in the cerebellum may have difficulty with movements of their arms or legs, including walking.  The brain stem controls the major life-sustaining automatic functions of the body, such as heart rate, breathing, blood pressure, and body temperature.  People with brain stem tumors may lose consciousness and need emergency medical care.  Spinal cord tumors most frequently occur in the cervical (neck) and thoracic (chest) areas of the spine.  The tumors may compress the spinal cord causing changes in sensation or muscle movement, as well as loss of bowel and bladder control. 
 
Additionally, tumors can cause a buildup of cerebral spinal fluid and increase the pressure inside of the skull.  Cerebral spinal fluid surrounds the delicate brain and spinal cord tissues and acts as a shock absorber during movement.  Increased intracranial pressure can cause headaches, nausea, vomiting, behavioral changes, and personality changes.

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Diagnosis
A doctor can diagnose hemangioblastoma by reviewing your medical records and conducting a physical examination, neurological examination, and some tests.  You should tell your doctor if other members of your family have hemangioblastoma or von Hippel-Lindau disease.  People with von Hippel-Lindau disease may develop this type of tumor in their retinas located in the back of the eye.  A detailed eye examination is used to identify tumors in that area.
 
Magnetic resonance imaging scans provide images of the structures in the brain and spinal cord.  They are useful for identifying the location and size of tumors.  Angiography is used to visualize the blood supply in the brain, spinal cord, and tumor.  There is no tumor grading system to categorize hemangioblastomas, as there is with other cancerous types of tumors.  However, doctors are able to identify the cell types in hemangioblastomas that are associated with a higher chance of recurrence.

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Treatment
Hemangioblastomas may be cured with surgical removal.  Endovascular embolization, a procedure that interrupts the blood supply to a tumor, may be used as a treatment alone or as a procedure to decrease the size of a tumor before surgery.  Traditional surgery, craniotomy, or radiosurgery may be used to remove the tumor.  Craniotomy involves removing a small portion of the skull to access the brain and remove the tumor.  Advanced stereotactic radiosurgery methods, such as Gamma Knife® or linear acceleration, target the tumor but spare the delicate surrounding nerve tissue.  Because radiosurgery methods do not use an incision, they are associated with a shorter recovery time and fewer complications than craniotomy.

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Prevention
People with hemangioblastomas should have careful followup.  People will be carefully monitored for tumor recurrence or new tumors.

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Am I at Risk
People with von Hippel-Lindau disease, an inherited condition, are at risk for hemangioblastomas.  People with von Hippel-Lindau disease may experience multiple tumors.  Otherwise, the tumors occur randomly among people with no known risk factors.  Hemangioblastomas occur in people of all ages, but they most frequently appear when people are in their thirties or forties.  Men are twice as likely to experience the condition as women are.

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Complications
Brain stem tumors are concerning because the brain stem controls the primary functions necessary for life.  People with brain stem tumors may experience significant neurological problems, coma, or death.

Hemangioblastomas carry a risk of bleeding.  Bleeding may cause neurological injury or damage if it compresses nerve tissue.

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Advancements
Researchers suspect that hemangioblastomas may be linked to other genetic abnormalities other than von Hippel-Lindau disease and continue to research such possibilities.

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This information is intended for educational and informational purposes only. It should not be used in place of an individual consultation or examination or replace the advice of your health care professional and should not be relied upon to determine diagnosis or course of treatment.

The iHealthSpot patient education library was written collaboratively by the iHealthSpot editorial team which includes Senior Medical Authors Dr. Mary Car-Blanchard, OTD/OTR/L and Valerie K. Clark, and the following editorial advisors: Steve Meadows, MD, Ernie F. Soto, DDS, Ronald J. Glatzer, MD, Jonathan Rosenberg, MD, Christopher M. Nolte, MD, David Applebaum, MD, Jonathan M. Tarrash, MD, and Paula Soto, RN/BSN. This content complies with the HONcode standard for trustworthy health information. The library commenced development on September 1, 2005 with the latest update/addition on April 13th, 2016. For information on iHealthSpot’s other services including medical website design, visit www.iHealthSpot.com.