A cavernous hemangioma is a mass that forms from a collection of malformed blood vessels. They’re usually present from birth. They go by several other names, including:

  • cavernous angioma
  • cavernous malformation
  • cavernoma

In this article, we’ll take a closer look at these vascular malformations, including where they develop, what causes them, and how they’re diagnosed and treated.

A word about medical terminology

The World Health Organization classifies cavernous hemangioma as a type of noncancerous blood vessel tumor in their International Classification of Diseases (ICD-11). However, the International Society for the Study of Vascular Anomalies (ISSVA) no longer uses the terms “carvernoma” or “cavernous hemangioma.” These terms are seen as outdated and misleading since they suggest that the mass is a tumor.

Vascular malformations are masses that form from collections of abnormal blood vessels. They’re estimated to occur in about 1% of people from birth. They can be further classified depending on their particular features.

Cavernous malformations are a specific type of vascular malformation made of widened blood vessels that create caverns through which blood travels very slowly. Their structure resembles a raspberry. They aren’t cancerous and have no potential for becoming cancer.

They most often develop in the brain but can also develop in other areas including the:

  • spinal cord
  • skin
  • retina
  • liver
  • adrenal glands
  • gastrointestinal tract

Cavernous malformations can change in size and number over time and can range from several millimeters to several centimeters in size. A millimeter is about the thickness of a credit card, and a centimeter is about the length of a staple.

As many as 40% of people with cavernous malformations in their brains never experience symptoms or receive a diagnosis. Most people in this group have:

  • a single malformation
  • no family history
  • no inherited mutation

People with a family history tend to develop multiple malformations and are more likely to have symptoms.

Symptoms vary depending on where the malformation forms.

Brain

Seizures are one of the most common symptoms of cavernous malformations in the brain. Other symptoms can include:

Spinal cord

A cavernous malformation of the spinal cord can cause limb:

  • numbness
  • weakness
  • paralysis
  • tingling
  • burning
  • itching

It can also cause loss of bladder control or bowel control.

Eye

A cavernous malformation can cause symptoms such as:

  • drooping eyelids
  • double vision
  • eye pain
  • vision problems from compression of the optic nerve

Symptoms are usually reversible after surgical removal.

Many people don’t experience any complications. However, a malformation in your brain can cause neurological symptoms if it bleeds. Complications depend on the area of your brain affected.

Cavernous malformations most commonly develop in the outer layer of your brain called the cortex. They can also develop in areas responsible for subconscious functions such as your cerebellum or brainstem.

Bleeding can lead to a hemorrhagic stroke and may be life threatening. According to the National Health Service (NHS), the yearly bleeding risk is about 2.4% per year. The risk is 0.3% to 2.8% per year if it hasn’t previously bled but increases to 6.3% to 32.2% if it has previously ruptured.

Cavernous malformations along your spine can cause neurological symptoms from spinal compression. If they develop in your eye, they can cause vision loss. Cavernous malformations occur in the eye in fewer than 1% of people with brain cavernous hemangioma.

It used to be thought that vascular malformations were present from birth, but it’s now known that they can also develop throughout life.

There are two forms of cavernous malformations: familial and sporadic. Familial malformations involve a gene inherited from one of your parents, and sporadic malformations develop without an inherited gene. About 80% of people have the sporadic form of the disease.

Familial cavernous malformation

People with a family history or multiple malformations are more likely to have the familial form due to mutations in one of the following genes:

  • CCM1 (KRIT1)
  • CCM2
  • CCM3 (PDCD10)

These genes are dominant, so you have a 50% chance of receiving one of these genes if one of your parents carries it.

New Mexico has an exceptionally high population of people with cavernous malformations. This is because the original Spanish settlers passed down a CCM1 gene for at least 14 generations.

Sporadic cavernous malformation

People with only one malformation are more likely to have the sporadic form. It’s thought that it’s caused by acquired genetic changes specifically in the cells of the affected blood vessels and isn’t passed between generations.

Cavernous malformation has been linked to brain radiation therapy in children.

A cavernous malformation can only be diagnosed with imaging or an autopsy.

Magnetic resonance imaging (MRI) is the standard diagnostic tool. Unless the malformation has recently bled, it won’t be detectable by a computed tomography (CT) scan or angiography.

Experts recommend genetic testing for people with a family history.

About 25% of people diagnosed with cavernous malformations are children.

Medications can treat specific symptoms such as seizures or headaches. Your doctor may recommend surgery if you’ve had recent bleeding.

Surgery to remove a cavernous malformation in your brain may be a traditional craniotomy where part of your skull is removed to access your brain. Radiosurgery is controversial but may be an option for single malformations that can’t be reached with traditional surgery. It involves very focused beams of radiation.

In a 2020 study, researchers found that half of a group of 12 children with bleeding and multiple malformations in their brains required additional surgery within 2.5 years. Of the nine participants without either of these risk factors, none needed additional surgery.

Your doctor may also recommend surgery to remove a cavernous malformation from other places such as your eye or spinal cord if they’re causing problems.

Many people with cavernous malformations in their brains never develop symptoms, and the outlook tends to be favorable. Symptoms are more likely to develop in people with large malformations.

Malformations in other places also tend to be favorable. Surgical resection of malformation of the spinal cord is associated with a good outlook, but in some cases, full recovery might not be possible.

Symptoms in people with an eye cavernous malformation are usually reversible after surgery.

Are cavernous hemangiomas life threatening?

Some cavernous malformations can be life threatening. Bleeding in the brain can cause a stroke. A stroke can cause permanent disability or death.

Can cavernous hemangiomas be prevented from developing?

It’s not clear whether cavernous malformations can be prevented. They’re thought to form due to genes inherited from your parents or genetic changes acquired throughout your life. Researchers are still trying to understand why these changes take place.

Are cavernous hemangiomas considered brain tumors?

The ISSVA no longer uses the term “cavernous hemangioma” or “cavernoma” because the malformations are not considered tumors. The suffix “-oma” indicates “tumor.”

Can cavernous hemangiomas of the eye/retina cause blindness?

Cavernous malformations of the retina usually don’t cause symptoms. However, vision loss can occur in the cases of macular malformations. Your macula is the part of your retina that’s responsible for your central vision.

A cavernous malformation is a raspberry-shaped mass of abnormal blood vessels that most often develops in the brain. Many people don’t experience any symptoms.

Individual symptoms such as seizures can be managed with medications and other noninvasive treatments. A doctor may recommend surgery to remove the malformation if it’s causing problems.