Rare brain cancers: What to know

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Atypical teratoid/rhabdoid tumors (AT/RTs) are a very rare form of brain cancer. Around 50% of these tumors begin in the cerebellum or the brainstem.

The cerebellum is the part of the brain responsible for:

• movement
• posture
• balance

The brainstem plays a role in the following functions:

• breathing
• seeing
• hearing
• walking
• heart rate
• talking
• eating

AT/RT tumors are fast-growing and often occur in children who are 3 years old or younger. This type of cancer can also occur in people who are older.

Outlook

People with AT/RT tumors often have a less favorable outlook than those with other central nervous system (CNS) embryonal tumors.

Factors that can affect a person’s outlook include:

• specific inherited gene changes
• how much of the tumor remains after treatment
• if the cancer has spread to other parts of the brain or body
• the person’s age

Learn more about rhabdoid tumors.

The choroid plexus is a structure that lines the brain’s ventricles. It helps produce cerebrospinal fluid (CSF).

Choroid plexus tumors develop in the choroid plexus. They often form within the brain’s ventricles. They may also form in other areas of the central nervous system (CNS).

These tumors most commonly occur in children during their first year of life. They can also affect older people.

Choroid plexus tumors are rare. Approximately 2,340 people living in the United States have this type of tumor.

Outlook

The 5-year relative survival rate of a type of cancer refers to the percentage of people who live for at least 5 years after they have received this diagnosis.

The 5-year relative survival rate among adults in the U.S. for choroid plexus tumors is 70.5%.

Factors that can affect a person’s outlook include their age and health when they develop the cancer and how the condition responds to treatment.

The jugular foramen is an opening in the base of the skull that allows essential structures, including nerves and arteries, to pass through.

Glomus jugulare tumors are a very rare form of paraganglioma, a type of neuroendocrine tumor. They affect approximately 0.07 per 100,000 people. This is around 1 case per 1.3 million people.

This type of slow-growing tumor develops in the jugular foramen. The majority of glomus jugulare tumors are benign (noncancerous), but approximately 1 to 5% are cancerous.

As they increase in size, these tumors can cause the temporal bone in the base of the skull to erode.

Outlook

The 5-year relative survival rate for people with a small paraganglioma that has not spread to other areas of the body is approximately 95%.

If the tumor grows back after surgery or has spread to other areas of the body, the 5-year relative survival rate is between 34% and 60%.

Endothelial cells make up the layer that lines blood vessels. These cells regulate the exchange of substances between the bloodstream and surrounding tissues.

Hemangiomas are benign tumors that develop when there is a buildup of endothelial cells.

If these tumors develop in the blood vessels of the brain, medical professionals may refer to them as brain cavernous angiomas.

This type of tumor most often occurs in adults, but 25% of cases appear in children.

Outlook

Asymptomatic brain cavernous angiomas may not require treatment.

A tumor that causes a person to experience symptoms may require surgery. Surgery may cause complications, which can lead to permanent neurological issues.

Neurons are specialized nerve cells in the CNS. They are conducting cells that send messages throughout the CNS and body.

Glial cells do not conduct nerve impulses like neurons. Instead, they help support and protect neurons.

Neuronal and glioneuronal tumors are rare tumors that develop either in the neurons or the neurons and glial cells.

They most commonly occur in young adults and children and make up less than 5% of primary CNS tumors.

Outlook

Glioneuronal and neuronal tumors are generally slow-growing.

A child with a low grade neuronal tumor may have a more favorable outlook if a surgeon can remove the tumor during surgery.

Children with high grade tumors of the glial cells may have a less favorable outlook.

In some cases, a younger child (under 1 year old) may have a high grade tumor of the glial cells that features certain fusion genes.

This can help improve their outlook compared with older children who have a high grade tumor of the glial cells but do not have these genetic changes.

The pineal gland is a gland in the brain that plays a role in the circadian cycle. This means it influences a person’s sleep and wakefulness.

Tumors in the pineal gland arise from stem cells near the gland and may spread to other areas of the body through the CSF.

Pineal tumors most often occur in children and are most common during the first 20 years of life.

They are very rare. Approximately 1,820 people have this type of tumor in the U.S.

Outlook

The 5-year relative survival rate for pineal tumors is 75.5%.

Factors that can indicate a less favorable outlook include when cancer spreads to nearby structures or when a person experiences extensive peritumoral edema, which is a buildup of fluid in the area around the tumor.

Diffuse midline glioma (DMG) is a form of brain cancer that develops in the glial cells of the midline structures in the CNS. These include the:

• thalamus
• brainstem
• spinal cord

These tumors are aggressive, fast-growing, and can spread to other areas of the body.

Outlook

People with DMG typically do not have a favorable outlook. These tumors often spread to other areas of the brain. This makes surgery to remove the tumors more difficult.

The average survival time after receiving a diagnosis is less than 1 year.

There are many different types of brain tumors that can cause a varying range of symptoms.

However, common symptoms of brain tumors include:

• headaches
• nausea and vomiting
• seizures
• changes in behavior
• vision problems, including blurred vision
• problems with balance
• drowsiness
• speech issues or difficulty understanding words
• weakness or numbness in parts of the body, often on one side
• hearing loss

Read more about the symptoms of brain tumors.

There are a number of possible treatment options for brain tumors, including:

• Surgery: A surgeon may remove the tumor or as much as they safely can without affecting the person’s brain function. They may also carry out surgery to take a biopsy or help prevent or treat symptoms or complications of brain cancer.
• Radiation therapy: Radiation therapy involves using high energy rays or small particles to kill cancer cells. It may be a main treatment option if others are not suitable. A person may also undergo radiation therapy to get rid of any remaining cancer cells left after surgery.
• Chemotherapy: Chemotherapy involves using anticancer drugs to kill cancer cells. A medical professional may administer the drugs directly into the CSF through a ventricular access catheter.
• Targeted drug therapy: Targeted drug therapy can specifically target and kill cancer cells in the brain. These drugs may work when chemotherapy treatments are not effective. Targeted therapy does not usually play an important role in brain cancer treatment but may be useful for treating specific tumor types.

Brain cancers occur when atypical groups of cells in the brain grow uncontrollably and multiply.

There are many different types of brain cancer. Common symptoms include headaches, nausea and vomiting, seizures, changes to personality, and vision problems.

Some rare forms of brain cancer include atypical teratoid/rhabdoid tumors, choroid plexus tumors, glomus jugulare tumors, and hemangiomas.

Common treatments for brain cancer include surgery, radiation therapy, and chemotherapy.