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Donations made to the Cristian Rivera Foundation go toward supporting medical facilities and scientific trials at the forefront of their field specifically researching cures for DIPG. Our foundation has pledged its support to Dr. Mark Souweidane of Memorial Sloan Kettering Cancer Center who is also the Director of the Weill Cornell Medical College Pediatric Brain and Spine Center.
Dr. Souweidane, inspired by his patient Cristian Rivera and other children affected by devastating childhood cancers, launched a pioneering FDA-approved clinical trial in 2012 that introduced a surgical component to the treatment of children with DIPG for the first time. The innovative use of Convection-Enhanced Delivery (CED) is the first of its kind to administer radio immunotherapy to children with an otherwise incurable tumor.
The Cristian Rivera Foundation also supports the research of Dr. Oren Becher of Duke University. His lab builds mouse models of DIPG tumors in order to better understand the makeup of these tumors, specifically the genetic alterations that are most important to inhibit therapeutically.
Diffuse Intrinsic Pontine Glioma (DIPG) is a malignant tumor found in the brain stem, the part of the brain that controls many vital functions. DIPG affects children of both sexes equally and is typically diagnosed in patients ages 5-9 and accounts for 10-15 percent of all childhood central nervous system (CNS) tumors. The prognosis is grim with survival beyond 12 months being rare.
There are no known factors or conditions that predispose an individual to the development of Diffuse Intrinsic Pontine Glioma.
DIPG tumors generally have a short duration (about 1 month) of symptoms prior to diagnosis, which reflects the rapid growth of these tumors. The following are the most common DIPG symptoms, however each child may experience symptoms differently.
Common symptoms may include:
• Rapidly developing inability to control eye movements, facial expressions, chewing and swallowing, due to problems in the cranial nerves
• Weakness in the arms and legs
• Impaired ability to walk
• Hydrocephalus (build-up of fluid pressure in the brain)
• Symptoms of increased pressure within the brain.
These symptoms include:
DIPG symptoms may resemble other conditions or medical problems. Always consult your child’s physician for a diagnosis.
Because these tumors grow so quickly, most patients develop symptoms over a couple of weeks. Each child may experience different symptoms, but common complaints
These symptoms typically lead to a CT or MRI of the brain. MRI is usually confirmatory and further diagnostic tests or biopsy are rarely needed.
Gliomas are heterogeneous tumors that are classified according to their most aggressive appearing elements. The World Health Organization classification scheme includes 4 grades of glioma. The pathological grade of DIPGs is not always established. Only 25-30 percent of these tumors are biopsied. Their clinical course is most consistent with their being the most aggressive form of astrocytic tumor or the grade IV glioblastoma multiforme.
These tumors are characterized as being of astrocytic origin but having increased numbers of cells (hypercellularity), abnormal cells and nuclei (cytologic and nuclear atypia), increased proliferation of cells (mitoses), increased cell death (necrosis) and increased growth of blood vessels (vascular endothelial proliferation). These are aggressive tumors that infiltrate nearby normal brain tissue and have a significant tendency to spread outside of the central nervous system.
A diagnosis of DIPG is commonly made from characteristic radiologic studies. The location of these tumors and their tendency to diffusely infiltrate normal tissue makes biopsy a high-risk procedure. Biopsies have been performed when the symptoms and other tests do not seem typical for DIPG.
Diagnostic procedures for DIPG may include:
• Physical examination
• Computerized Tomography scan (also called a CT or CAT scan) – a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. CT scans are more detailed than general x-rays.
A CT scan can assess the density of tumor tissue, compared to normal brain tissue, as well as establish its mass effect (the effect of an additional space occupying mass within the closed cavity of the skull on the normal brain). DIPGs most often appear as round masses that expand the pons. When intravenous dyes are injected, these tumors avidly absorb dye and therefore are referred to as enhancing lesions.
• Magnetic Resonance Imaging (MRI) – a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
An MRI provides greater anatomical detail than a CT scan and can better distinguish between a tumor, tumor-related swelling and normal tissue. The typical MRI appearance of a diffuse intrinsic pontine glioma is a round mass that expands the pons. When intravenous dye is administered, it displays a ring of enhancement. There can be axial growth, which is growth up or down the brainstem, or exophytic growth which is growth forward or backward out of the brainstem.
• Magnetic Resonance Spectroscopy (MRS) – a test done along with an MRI at specialized facilities that can detect the presence of particular organic compounds produced by the body’s metabolism within sample tissue that can identify tissue as normal or tumor and may be able to distinguish between glial tumors and tumors of neuronal origin.
If a child has hydrocephalus, which is only present in about 1/10 children at the time of diagnosis, then surgical treatment is needed (shunt placement or third ventriculostomy). These treatments almost immediately result in some improvement in symptoms.
The use of steroids (dexamethasone) taken by mouth are commonly started after diagnosis and result in some improvement of symptoms.
Surgical biopsy, while possible, is seldom necessary to establish a diagnosis. A biopsy is very definitive for tumor and molecular/genomic characterization. A biopsy may also be necessary for inclusion in some clinical trials. Unfortunately DIPG is not amenable to surgical removal, owing to its highly invasive growth pattern. Radiation therapy usually involves 5 weeks of outpatient treatments. This is the mainstay of treatment. Radiation therapy commonly results in improvement of the symptoms and MR appearance of the tumor. Unfortunately this benefit is only transient and tumor recurrence is the rule after several weeks to months. Thus far, no chemotherapy regimen has been able to increase survival in children. Early phase clinical trials are currently focusing on novel therapy approaches, including molecularly-targeted therapy, epigenetic/histone modification agents, and local delivery strategies, including convention enhanced delivery (CED) and intra-arterial (IA) delivery.
Side effects of DIPG treatment may arise from radiation and chemotherapy. Radiation therapy often produces inflammation, which can temporarily exacerbate symptoms and dysfunction. To control this inflammation steroids are sometimes necessary.
Some of the chemotherapy agents are associated with fatigue, diarrhea, constipation and headache. These side effects can be effectively managed under most circumstances with standard medical approaches.
Many specialized brain tumor treatment centers have now added staff who are experts in complementary or alternative medicine. These treatments, including acupuncture/acupressure, therapeutic touch, massage, herbs and dietary recommendations, can also help to control pain and side effects of therapy.
The prognosis for DIPG is abysmal. Median survival is about 1 year. Only 1 out of 10 children may survive past 2 years.
Clinical trials and experimental therapies are available for patients with relapsed high-grade gliomas at specialized centers. Current trials include novel medications as well as new methods for the delivery of more traditional agents.
For more information visit: weillcornellbrainandspine.org/condition/diffuse-intrinsic-pontine-glioma-dipg