Diffuse Intrinsic Pontine Glioma (DIPG)

What is DIPG?

DIPG is a type of tumor that starts in the brain stem, the part of the brain just above the back of the neck and connected to the spine. The brain stem controls breathing, heart rate and the nerves and muscles that help us see, hear, walk, talk and eat. These tumors are called gliomas because they grow from glial cells, a type of supportive cell in the brain.

What are the stages of DIPG?

DIPG falls into the Glioma staging system, so they can be classified according to the four stages below based on how the cells look under the microscope. The grades are from the least severe to the most severe.

• Low Grade: Grade I or II means that the tumor cells are the closest to normal.
• High Grade: Grade III or IV means that these are the most aggressive tumors.

The main issue with DIPG is that most of these tumors are not classified by grade because surgery to obtain tissue by biopsy or to remove the tumor is not safe because of the location of the tumor. When these tumors are biopsied, they are usually grade III or grade IV tumors, which tend to behave very aggressively. Most of the tumors are diagnosed by their appearance on MRI.

How common is DIPG?

• About 10% to 20% of all childhood brain tumors are DIPG or brainstem gliomas.
• They are more common in children between the ages of 5 and 10 years, but can occur at any age in childhood. Though more rare, they can also occur in adults.

What are the symptoms of DIPG?

Symptoms usually develop rapidly in the majority of patients because of the fast growth of these tumors.

The most common symptoms related to DIPG include the following:

• Problems with balance and walking
• Problems with the eyes (including double vision, drooping eyelids, uncontrolled eye movements, blurred vision)
• Problems with chewing and swallowing
• Nausea and vomiting
• Morning headache or headache that gets better after the child vomits
• Facial weakness or drooping (usually one side)

How is DIPG treated?

• Radiation therapy — currently, is the primary therapy for newly diagnosed DIPG in children older than 3. It uses high-energy X-rays from a specialized machine or other types of radiation to kill cancer cells and shrink tumors (stop them from growing). Radiation provides a temporary response in most patients but does not provide a cure.
• Chemotherapy — is being used along with radiation therapy and other biologic agents in multiple trials to decide if we can find ways to improve the survival rates of patients with DIPG.
• Surgery — is rarely used to diagnose DIPG because of the risks involved in surgery in this area of the brain. There are cases where a biopsy can be obtained safely, but the use of surgery to remove the tumor is very rare.
  • A biopsy (removal of affected tissue to be examined) may be done to decide the tumor’s type and grade.
  • If the biopsy finds cancer cells and the patient is still in surgery, the surgeon may take out as much tumor as can be removed safely. Depending on where the tumor is located, this might not be an option.

What are the survival rates for DIPG?

Unfortunately, the survival rate for DIPG remains very low. At this time there is no cure for this tumor. The brain tumor team at St. Jude along with colleagues at many other pediatric brain tumor programs across the U.S. will continue to strive toward finding a cure for patients with DIPG.

Why choose St. Jude for your child’s DIPG treatment?

• St. Jude has one of the largest pediatric brain tumor programs in the country.
• The St. Jude staff is dedicated to treating children who have serious illnesses. Here, you will find a hospital and staff that has only one focus—your child.
• St. Jude is committed to not only finding cures, but also helping children hold on to their quality of life. Children are not just small people. Their bodies and systems are unique, along with the disorders that affect them. For the best care, they need a specialized team. That is what you get from the St. Jude Brain Tumor Program.
• By working closely with the pediatric neurosurgeons at Le Bonheur Children’s Hospital in Memphis, the St. Jude Brain Tumor Program offers an expert team of doctors from many medical fields. The team includes: neuro-oncologists, who study and treat cancer in the brain and nervous system; neurosurgeons, who operate on the brain, skull, scalp, and spinal cord; and radiation oncologists, who treat cancer with radiation. All of these doctors have devoted their careers to finding cures and providing treatment for children with brain tumors. This diverse knowledge is vital to the complete care of children with brain tumors.
• Our brain tumor doctors also work closely with support staff in nursing, rehabilitation services, neuropathology, pharmacy, neuroradiology, neuropsychology, clinical nutrition, child life and social work, along with many others. Support staff members have been specially trained to care for children with pediatric brain tumors.
• The nurse-to-patient ratio at St. Jude is unmatched— averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
• The St. Jude Brain Tumor Program has made great advances in treatments for brain tumor patients. Some of the world’s best neuroscientists, cancer biologists and doctors from the St. Jude Neurobiology and Brain Tumor Divisions combine their efforts to seek cures. In this interactive program, discoveries about the biology of both normal and harmful cell growth are rapidly tested in clinic studies. These studies include Phase I, II and III trials of the most common and deadly forms of childhood brain tumors.
• Being able to take a drug directly from the lab to your child is one of the major advantages of centers like St. Jude. Before treating a patient with any new drug, the St. Jude staff does many tests in the lab. The tests show if the drug is safe, if it works, and the best way to use it to help patients.
• St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
• Along with the treatment studies being constantly developed at St. Jude, the staff also treats patients on studies developed by the Children’s Oncology Group, the Pediatric Brain Tumor Consortium and the Pacific Pediatric Neuro-Oncology Consortium. Many St. Jude staff members play key roles in developing these studies, which are large tests carried out at centers across the United States.
• St. Jude practices patient family-centered care. Patient family-centered care is health care that focuses on the family as a child’s primary source of strength, support and well-being. At the heart of patient family-centered care is the belief that health care staff and the family are partners, working together to best meet the needs of the child. Excellence in health care happens when we work together and honor the expertise each of us brings to every health care encounter.
• St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.