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Rare Brain Tumors

Overview of Rare Brain Tumors

A brain tumor is a mass of abnormal cells in the brain. In the United States, a condition is defined as rare when it affects fewer than 200,000 people.

More than 150 different types of brain tumors have been documented, but they can be divided into two broad categories: primary and secondary. Primary brain tumors originate in the brain, while secondary (metastatic) tumors spread to the brain from elsewhere in the body.

Secondary tumors are malignant (cancerous), but primary tumors can be malignant or benign (noncancerous).

Types of rare brain tumors

Gliomas represent the majority of primary brain tumors. These tumors arise from supporting cells of the brain called glia. Gliomas include astrocytomas, ependymomas, glioblastomas, medulloblastomas, and oligodendrogliomas.

Non-glial tumors may consist of nerve cells (neurons), blood vessels, or glands. Some tumors may consist of both glial and nerve cells.

Atypical teratoid/rhabdoid tumor (AT/RT)

Atypical teratoid/rhabdoid tumors are fast-growing, cancerous tumors that originate from embryonal cells in the brain. These cells include rhabdoid, neuroepithelial, epithelial, and mesenchymal. Because AT/RTs grow rapidly, symptoms tend to worsen quickly. Most of these tumors are caused by changes in the SMARCB1 (INI1) gene. AT/RTs occur more often in children but can also occur in adults.

Choroid plexus tumor

Choroid plexus tumors originate in the choroid plexus—a structure that lines the fluid-filled cavities in the brain, known as ventricles. They may be cancerous or noncancerous. Choroid plexus tumors can cause cerebrospinal fluid (CSF) to build up inside the ventricles and increase pressure within the skull, a condition known as hydrocephalus. These tumors are more common in children but can occur in adults.

Glomus jugulare tumor (paraganglioma)

Glomus jugulare tumors arise from the temporal bone in the skull, known as the jugular foramen. This region contains nerve fibers, known as glomus bodies, and is where the jugular vein leaves the skull. Glomus jugulare tumors can affect the ear, upper neck, base of the skull, and surrounding blood vessels and nerves. They are rarely cancerous, but treatment may be needed to relieve symptoms such as hearing problems, facial nerve weakness, or dizziness. They tend to occur later in life, usually around age 60 or 70.


Hemangiomas are tumors made up of blood vessels. Hemangioblastomas arise from the cells lining the blood vessels, while hemangiopericytomas originate from the cells surrounding the blood vessels in the meninges—the coverings of the brain and spinal cord. Hemangioblastomas are benign and slow-growing, while hemangiopericytomas can spread to other parts of the body.

Neuronal and glioneuronal tumors

Neuronal tumors develop from nerve cells, rather than glial cells. Nerve cells, or neurons, are involved in communication in the brain, while glial cells insulate and support the functions of neurons. Glioneuronal tumors, such as dysembryplastic neuroepithelial tumors (DNETs), include both nerve cells and glial cells. Neuronal and mixed neuronal-glial tumors are typically small and benign, but they can cause problems when they press against important brain structures. Anaplastic ganglioglioma is a subtype of neuronal tumor that grows more quickly than most neuronal tumors.

Pineal tumors

Pineal tumors develop from cells in the pineal gland, which is located in the center of the brain and involved in hormone secretion. They include pineocytomas, pineoblastomas, and mixed pineal tumors. Some tumors that develop in the pineal region may not actually involve the pineal gland itself and, therefore, are not true pineal cell tumors. These include germinomas, non-germinomas, meningiomas, astrocytomas, gangliogliomas, and dermoid cysts.

Rare Brain Tumor Symptoms

Brain tumor symptoms can vary depending on the type, location, size, and growth rate of the tumor.

General symptoms of brain tumors include:

  • Confusion
  • Difficulty thinking, speaking, or articulating
  • Dizziness
  • Facial numbness or tingling
  • Headaches, (new onset, change in pattern, more frequent, more severe, etc.)
  • Hearing changes
  • Loss of balance
  • Nausea or vomiting
  • Personality or behavior changes
  • Seizures (new onset)
  • Vision changes
  • Weakness or paralysis in one part or on one side of the body

If you are experiencing any symptoms, please contact a medical professional.

Rare Brain Tumor Treatments

Treatment for a brain tumor depends on its type, size, location, and growth rate, as well as your overall health. A combination of treatments may be used.

Possible treatments include:

  • Chemotherapy
  • Immunotherapies
  • Radiation therapy
  • Radiosurgery, such as Gamma Knife or CyberKnife (available here at Barrow)
  • Surgery
  • Targeted Therapies
  • Tumor-Treating Fields

Neuro-rehabilitation may be necessary following treatment if the tumor affected regions of the brain that control motor skills, speech, vision, or thinking.

Individualized Care

There is no single recipe, or “cookbook” approach, that works best for everyone with a brain tumor. Every brain tumor is unique, as is each patient. Personalized medicine approaches, such as tumor profiling to look for specific gene mutations, can help determine the best therapies available for you.

Quality of Life Considerations for Rare Brain Tumors

At Barrow, we believe brain tumor treatment should be focused on optimizing quality of rather than simply extending life. Our neuro-rehabilitation specialists can help you maximize your independence and return to a fulfilling life with renewed self-esteem.

We also offer a Brain Cancer Survivorship Program to foster relationships between families who have been affected by brain tumors and provide ongoing support.

Additional Information on Rare Brain Tumors

How are brain tumors diagnosed?

The following tests may be used to diagnose a brain tumor and determine its type:

  • Biopsy/resection
  • Computed tomography (CT) scan
  • Positron emission tomography (PET) scan
  • Magnetic resonance imaging (MRI) scan
  • Neurological exam

Information and Resources

Group 49
Nearly 700,000 people in the U.S. are living with a brain tumor.

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