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Diffuse intrinsic pontine glioma, or DIPG, is an aggressive brain tumor. It forms in the base of the brain. DIPG begins in the brainstem. The area is called the pons.
The pons controls vital life functions including:
Nerves that control vision, hearing, speech, swallowing, and movement also pass through this part of the brain.
About 200–300 new cases of DIPG are diagnosed every year in the United States. DIPG most often occurs in children ages 5–10 years old. But it can occur in younger children and teens. DIPG is rare in adults.
DIPG grows from glial cells. These cells make up the supportive tissue of the brain. DIPG is a diffuse tumor. This means it is not well-defined or contained. The tumor extends finger-like projections into healthy tissue.
Because of the location in the brainstem and spread-out nature of the tumor, surgery cannot safely remove DIPG.
DIPG is very hard to treat. Most children do not survive more than 2 years after diagnosis. The main treatment for DIPG is radiation therapy. Radiation temporarily improves symptoms in most patients, but it is not a cure. Families may consider clinical trials that test new treatments to see if outcomes can be improved.
Early palliative care is important. It can help families manage symptoms and promote quality of life.
DIPG tumors grow quickly. Symptoms usually develop in a short period of time (often about 1 month before diagnosis). There is a rapid onset and fast progression. Signs and symptoms of DIPG may include:
Less common symptoms may include:
Doctors look for key features of the tumor on the MRI to diagnose DIPG:
About 10–20% of pediatric brain tumors are found in the brainstem. When a tumor develops in the brainstem, it is usually DIPG. But about 20% of brainstem tumors are low-grade astrocytomas. These are not considered DIPG.
Doctors sometimes use a biopsy to diagnose DIPG. In the past, a biopsy was not usually used. Reasons for not doing a biopsy include:
With better surgical techniques and advances in understanding, biopsy has become more common for DIPG. After a biopsy, a pathologist will look at the tissue sample under a microscope to identify the specific type and grade of tumor.
The tumor will also be tested for genetic changes or markers. Understanding tumor histology and molecular features may one day offer better treatments for DIPG. These could include targeted therapy and immunotherapy.
No one knows the cause of DIPG. But scientists are learning more about gene changes linked to these tumors. Having certain genetic disorders, including neurofibromatosis type 1 (NF1), may increase the risk of a brainstem glioma.
There is no standard staging system for DIPG. Treatment is based on 2 main factors:
Gliomas are grouped by how they look under the microscope. The more abnormal the cells look, the higher the grade. Grade 1 and 2 tumors are low-grade gliomas. The cells look more like normal cells and grow more slowly.
Grade 3 and 4 tumors are high-grade gliomas. They are aggressive and grow quickly. These tumors can spread throughout the brain.
DIPG tumors are usually high grade. Rarely, DIPG may appear as a low-grade tumor (grade 2).
DIPG is quite aggressive. No cure is known at this time. The standard of care is based on radiation therapy.
Much of DIPG patient care focuses on controlling symptoms and supporting quality of life. Corticosteroid medicines such as dexamethasone can help reduce some of the tumor’s symptoms. These medicines are usually used at diagnosis and at tumor progression to help manage neurologic symptoms.
A number of clinical trials are exploring therapies that may improve outcomes for patients with DIPG.
Many patients with DIPG get care through a clinical trial. Clinical trials are available:
Many times, the tumor may shrink for a while after initial radiation therapy. Symptoms improve, and patients can go home to their usual activities. This is sometimes called the “honeymoon period.”
This phase is hard to predict. Some children are almost back to normal during this time. Some children do not improve at all. Families may use the time to be together or do something special as a family. Wish-granting organizations can often help with this.
DIPG has no cure at this time.
Fewer than 10% of children in the United States survive more than 2 years after diagnosis. A somewhat better outcome may be seen in very young patients (3 years old or younger) and those who have symptoms for a longer time before diagnosis.
Patients with neurofibromatosis type 1 (NF1) who have diffuse brainstem tumors may also live longer with the disease.
Long-term survival in DIPG is generally linked to atypical features or misdiagnosis (the tumor was not actually DIPG).
In DIPG, symptoms get worse over time. Supportive care helps maintain quality of life as much as possible for as long as possible.
Talk to your care team about problems to expect and ways to help manage them.
Symptoms can worsen rapidly when DIPG comes back. But symptoms and their course and severity can vary.
Some signs and symptoms of late-stage DIPG include:
Medicines can help control pain, nausea and vomiting, anxiety and depression, and medical problems that develop as the tumor grows.
Art therapy, music therapy, and other complementary therapies can also help patients and families manage symptoms.
This is usually not due to the tumor itself. Weight gain, face swelling, and puffiness are mainly due to high doses of corticosteroids given to help manage symptoms of DIPG.
Brain tumors can cause fluid and pressure to build up. This pressure (hydrocephalus) causes symptoms such as headaches, nausea, weakness, and problems walking. Steroid medicines like dexamethasone decrease brain swelling and pressure. But they also have side effects, especially when given in high doses or for a long time.
Corticosteroids can increase appetite and cause patients to eat more and gain weight. These medicines can make it hard for the body to get rid of fluids, causing swelling. The body may also store fat differently. This can lead to puffy cheeks or “moon face.”
Rapid weight gain sometimes causes stretch marks on the skin. The change in appearance due to corticosteroids can be distressing to patients and families. Other side effects of steroid treatment include mood swings, irritability, and muscle weakness.
Despite the side effects, these medicines are an important part of supportive care and quality of life for many brain tumor patients. Talk to your care team about side effects and goals of care.
Read more about steroids from the American Brain Tumor Association.
With the poor prognosis of DIPG, it is important for families to talk with their care teams about goals of therapy, palliative care, and end of life care. These conversations should begin early in the care process.
Goals of care change over the course of the disease with the changing needs of the patient and family.
A team of health care providers can help meet specific patient needs as DIPG progresses.
Palliative care or quality of life services help patients and families:
Hospice care provides medical and practical support as patients near the end of life. Rehabilitation services provide care for movement, speech, hearing, or swallowing problems. Child life, social work, spiritual care, and psychology can help with emotional and practical needs through the cancer journey for the entire family.
Reviewed: May 2023