Diffuse midline glioma (DMG)

Symptoms

Symptoms of diffuse midline glioma (DMG) depend on where in the central nervous system the tumor is growing. Symptoms at first may be mild and easy to overlook. But they get worse over a few weeks or months. It's very difficult to diagnose DMG in the early stages.

Common symptoms of DMG include:

• Drooping on one side of the face.
• Weakness in the facial muscles.
• Weakness in the arms and legs, particularly on one side.
• Clumsiness.
• Fatigue.
• Headaches, particularly in the morning.
• Nausea and vomiting.
• Problems with speech or swallowing.
• Balance problems.
• Difficulty walking.
• Tingling or numbness, which may affect the face, arms, hands or legs.
• Difficulty concentrating.
• Seizures.
• Back pain.
• Changes in bladder and bowel function.
• Vision problems, including blurry or double vision.
• Hearing problems, including loss of hearing.
• Behavior changes, such as irritability or trouble at school.
• Gaining weight.

When to seek care

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

Risk factors

There are no known risk factors for diffuse midline glioma and no known way to prevent these tumors.

Treatment

Diffuse midline glioma (DMG), is a very aggressive brain tumor that spreads very quickly. There isn't a cure for DMG, but treatments can help manage symptoms so that people with DMG can live longer and more comfortably.

Radiation therapy

The most common treatment for DMG is radiation therapy. Radiation therapy uses precise, highly concentrated beams of energy to destroy the tumor cells. This treatment usually is given in multiple sessions over about six weeks. It can slow down the growth of the tumor and help reduce some of the symptoms it causes.

Unfortunately, DMG tumors often grow back after radiation therapy.

Surgery

Usually, surgery isn't an option for these types of tumors because of where they grow in the brain. They grow in the midline, where essential parts of the brain are found. For this reason, surgery can be too risky. Also, the poorly defined edges of the tumor make it difficult to remove with surgery.

Medicines

Chemotherapy, which is medicine designed to kill cancer cells, usually isn't effective for DMG. But sometimes, chemotherapy may be used along with radiation therapy.

Palliative treatment

Palliative treatment does not aim to cure cancer but to make life better and manage symptoms such as difficulty swallowing, pain, headaches, and difficulty walking and moving around. Palliative treatment for DMG can include:

• Steroid medicines to reduce swelling in the brain.
• Support for feeding and nutrition.
• Speech therapy.
• Occupational therapy.
• Physical therapy.
• Medicines to prevent seizures.
• Pain medicine.
• Procedures to drain fluid buildup on the brain.
• Counseling and other therapy to help cope with DMG.

Potential future treatments

Researchers are studying new medicines to treat diffuse midline glioma and new ways of delivering them. Research includes new chemotherapy medicines that target the changed genes in DMG tumors.

Clinical trials are studying medicines that target these cancer cells specifically and deliver them directly to the tumor via a catheter and other novel methods.

Clinical trials also are looking at immunotherapy treatments for DMG. Immunotherapy medicines help the body's immune system find and destroy cancer cells. These medicines include immune checkpoint inhibitors, monoclonal antibodies and vaccines. All of these treatments target cancer cells through the immune system.

Current clinical trials are investigating the use of chimeric antigen receptor (CAR)-T cell therapy. In this treatment, white blood cells called T cells are removed from the blood. In a lab, scientists change these T cells and add a receptor called a CAR. The CAR allows the T cells to track and find cancer cells more easily.

Scientists then grow and multiply the CAR-T cells in a lab, producing millions of very powerful cells. These CAR-T cells are put back into the blood through a needle placed in a vein in the arm, also called an IV. CAR T-cells can keep living and working in the body for a long time, so they're sometimes called a "living drug."

Prognosis

Every person with DMG and every diagnosis is different. DMG is usually fatal. Life expectancy is usually between 9 and 12 months after diagnosis. Most people survive less than a year after diagnosis. Some people live longer with DMG. This depends on several factors, including how well they respond to treatment and the tumor grade at diagnosis. Clinical trials can help people with DMG live longer.

A healthcare team can provide support and guidance for end-of-life care.

Preparing for an appointment

If you have symptoms that worry you, make an appointment with a doctor or other healthcare professional. If you have DMG, you'll likely be referred to specialists, such as:

• Doctors who specialize in brain disorders, called neurologists.
• Doctors who treat cancer with medicine, called medical oncologists.
• Doctors who use radiation to treat cancer, called radiation oncologists.
• Doctors who specialize in nervous system cancers, called neuro-oncologists.
• Surgeons who operate on the brain and nervous system, called neurosurgeons.
• Rehabilitation specialists.

Here's some information to help you get ready for your appointment.

What you can do

• Be aware of anything you need to do before your appointment. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as change your diet.
• Write down any symptoms you've been experiencing and how long you've had them.
• List your key medical information, including all conditions you have and the names of any medicines you're taking. Include prescription medicines and medicines you can buy without a prescription.
• Take a family member or friend with you. Sometimes it can be difficult to remember all of the information you get during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare professional.

Questions to ask at your first appointment include:

• What may be causing my symptoms?
• Are there any other possible causes?
• What kinds of tests do I need? Do these tests require any special preparation?
• What do you recommend for the next steps in determining my diagnosis and treatment?
• Should I see a specialist?

Questions to ask an oncologist or a neurologist include:

• Do I have DMG?
• How will the DMG grow over time?
• Where is the DMG located?
• How large is the DMG?
• What might happen because of the DMG?
• What are the goals of my treatment?
• Is surgery a treatment option? What are the risks?
• Is radiation therapy a treatment option? What are the risks?
• Will I need chemotherapy?
• What treatment approach do you recommend?
• If the first treatment doesn't work, what will we try next?
• What is the prognosis for my condition?
• Do I need a second opinion?
• Is there a clinical trial available for people with DMG? Is it right for me?

In addition to the questions that you've prepared, don't hesitate to ask any other questions that may come up during your appointment.

What to expect from your doctor

Be prepared to answer questions about your symptoms and your health history. Questions may include:

• What are your symptoms?
• When did you first notice these symptoms?
• Have your symptoms gotten worse over time?
• If you have pain, where does the pain seem to start?
• Does the pain spread to other parts of your body?
• Have you participated in any activities that might explain the pain, such as a new exercise or a long stretch of gardening?
• Have you experienced any weakness or numbness in your legs?
• Have you had any difficulty walking?
• Have you had any changes in your bladder or bowel function?
• Have you been diagnosed with any other medical conditions?
• Are you currently taking any medicines, including prescription medicines and medicines that you can get without a prescription?
• Do you have any family history of noncancerous or cancerous tumors?