Diffuse Intrinsic Pontine Glioma

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2. Where does my donation go?

Donations made to the Cristian Rivera Foundation go toward helping DIPG families in need and supporting medical facilities and scientific trials at the forefront of their field who are specifically researching cures for Pontine Glioma. The Cristian Rivera Foundation has pledged its support to Dr. Mark Souweidane of Memorial Sloan Kettering Cancer Center and the Director of the Weill Cornell Medical College Pediatric Brain and Spine Center. Dr. Souweidane, inspired by his patient Cristian Rivera and other children and their families 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 Pontine Glioma 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 Pontine Glioma tumors in order to better understand the makeup of these tumors, specifically the genetic alterations that are most important to inhibit therapeutically.

3. What is a Diffuse Pontine Glioma (Brainstem Glioma)?

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 age 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.

4. What Causes Diffuse Pontine Glioma?

There are no known factors or conditions that predispose an individual to the development of diffuse pontine gliomas.

5. What are the Symptoms of a DIPG?

Diffuse pontine gliomas generally have a short duration (median 1 month) of symptoms prior to diagnosis, reflecting 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:

o headache (generally upon awakening in the morning)

o vomiting

o fatigue

DIPG symptoms may resemble other conditions or medical problems. Always consult your child’s physician for a diagnosis.

6. How is Diffuse Pontine Glioma Detected?

Because these tumors grow so quickly, most patients
develop symptoms over a couple of weeks. Each child may experience different symptoms, but common complaints

  • Double vision with crossed- eye movements and asymmetric facial expressions
  • Weakness in the arm and leg on one side of the body
  • Impaired ability to walk and loss of balance
  • Headache, fatigue, or vomiting, most commonly in the morning are typical symptoms of hydrocephalus (build-up of fluid pressure in the brain)

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.

7. How are Diffuse Pontine Gliomas Classified?

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 diffuse pontine gliomas 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 adjacent normal brain tissue and have a significant tendency to spread outside of the central nervous system.

A diagnosis of diffuse pontine glioma is most 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 diffuse pontine glioma. Diagnostic procedures for diffuse pontine glioma 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). Diffuse pontine gliomas 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. MRI provides greater anatomical detail than CT scan and can better distinguish between tumor, tumor-related swelling and normal tissue. The typical MRI appearance of a diffuse pontine glioma is a round mass that expands the pons. When intravenous dye is administered, it displays a ring of enhancement. There can be growth up or down the brainstem, axial growth, or growth forward or backward out of the brainstem, exophytic growth.

• magnetic resonance spectroscopy (MRS) - a test done along with 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.

8. What are the treatments for a Diffuse Pontine Glioma?

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.

9. How are Side Effects Managed?

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.

10. What is the expected outcome after treatment for Diffuse Pontine Glioma?

The prognosis for DIPG is abysmal. Median survival is about 1 year. Only 1 out of 10 children may survive past 2 years.

11. What About Progressive or Recurrent Disease?

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.

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