Is hemangioblastoma hereditary?

Hemangioblastomas are tumors that can form in the brain, spinal cord, root of the nerves and retina. Hemangioblastomas may be hereditary or may occur sporadically (without association with known genetic factors).

What is the difference between a hemangioma and a hemangioblastoma?

Spinal hemangioblastomas are usually located on the surface of the spinal cord. They account for 5-10% of intramedullary tumors, or tumors that arise on or in the spinal cord. Hemangioblastomas are one kind of hemangioma, or tumors of blood vessels.

Are hemangioblastomas malignant?

Hemangioblastomas grow in the blood vessels of the brain, spinal cord, and retina. These tumors can be caught early and removed before they grow too big. The tumors are benign. That means they will not become cancerous.

Are hemangioblastomas rare?

Hemangioblastomas make up about 2 percent of all primary brain tumors and about 3 percent of all spinal tumors. These slow-growing tumors can often be treated before any lasting complications arise.

How common is hemangioblastoma?

Hemangioblastomas are rare, accounting for only three percent of all spinal tumors. Most occur as a single tumor, but some patients develop hemangioblastomas as part of a genetic cancer syndrome called von Hippel-Lindau (VHL) disease.

Is VHL hereditary?

Because VHL syndrome is genetic, there is a chance that your relatives may have the mutation as well. Eighty percent of cases are inherited from a parent who has VHL syndrome.

What is the treatment for hemangioblastoma?

Symptomatic cerebellar or brainstem hemangioblastomas are typically treated by surgical removal through a sub-occipital or Retromastoid Craniotomy. The goal of hemangioblastoma surgery is to remove the enhancing tumor nodule completely which is able to be accomplished in 80-90% of patients.

Why is VHL autosomal dominant?

VHL disease is an autosomal dominant disorder resulting from a deletion or mutation in the VHL gene located on the short arm of chromosome 3. Each child of a person with VHL is at 50% risk of inheriting the altered copy of the gene.

How fast do hemangioblastomas grow?

Hemangioblastomas grew in a stuttering pattern. (mean growth period 13 +/- 15 months, mean quiescent period 25 +/- 19 months).

What is cerebellar hemangioblastoma?

Listen to pronunciation. (SAYR-eh-BEH-ler hee-MAN-jee-oh-blas-TOH-muh) A benign, slow-growing tumor in the cerebellum (part of the brain at the back of the head), made up of abnormal blood vessel growth.

What is the genetic hallmark of hemangioblastoma?

Although the exact cause of hemangioblastoma is unknown, its presence in various clinical syndromes may suggest an underlying genetic abnormality. The genetic hallmark of hemangioblastomas is the loss of function of the VHL gene. GeneTests lists the names of laboratories that are performing genetic testing for von Hippel-Lindau syndrome.

How often do hemangioblastomas occur?

Most hemangioblastomas occur sporadically. However, some people develop hemangioblastomas as part of a genetic syndrome called von Hippel-Lindau syndrome. These people usually develop multiple tumors within the brain and spinal cord over their lifetime.

Is hemangioblastoma cancer aggressive?

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.

What are the symptoms of hemangioblastomas?

This tumor accounts for about 2% of brain tumors. As it enlarges, it presses on the brain and can cause neurological symptoms, such as headaches, weakness, sensory loss, balance and coordination problems, and/or hydrocephalus (a buildup of spinal fluid in the brain). Most hemangioblastomas occur sporadically.