IN SHORT: Diffuse intrinsic pontine glioma (DIPG) is a brain tumor found in a part of the brain stem called the pons. The pons controls essential bodily functions such as heartbeat, breathing, swallowing, eye movement, eyesight, and balance. DIPG affects children almost exclusively. These children are typically between the ages of 4 and 11. DIPG is an aggressive tumor that interferes with all bodily functions, depriving a child of the ability to move, to communicate, and even to eat and drink. As a DIPG tumor begins to grow, it puts pressure on the nerves that control the essential bodily functions regulated by the pons. Children with DIPG commonly experience double vision, reduced eye movement, facial weakness or asymmetry, and arm and leg weakness. They also have problems with walking, coordination, speech, chewing, and swallowing. As the tumor progresses, it also interferes with breathing and heartbeat, which ultimately results in the child’s death.
Unlike many brain tumors, a DIPG tumor cannot be removed through surgery. Attempting surgical removal of a brain stem tumor would likely cause severe neurological damage and could even be fatal.Surgery is not an option for two reasons. First, because the pons is located in the center of the brain, a surgeon could not get to the tumor without damaging the surrounding portions of the brain. Second, even if the tumor could be accessed, total removal would be impossible. A DIPG tumor is not a solid, well-defined mass like some tumors. Instead, the tumor cells spread out between the healthy brain cells. Because the tumor cells could not be entirely removed without also removing the healthy cells, even if surgery were performed, the remaining cells would continue to divide and the tumor would soon return.
Radiation is part of the standard course of treatment for DIPG patients, as it is the only form of treatment that has proven benefits. For roughly 70% of DIPG patients, radiation causes the tumor to shrink, which provides relief from many of the symptoms associated with DIPG. The benefits of radiation, however, are only temporary. Even for those patients whose tumors shrink during radiation, the treatment does not increase their chances of survival, and the tumor begins to grow again within months. Because DIPG patients who do not undergo radiation have a median survival length of roughly 5 months, radiation therapy typically extends a patient’s life by about 3 months. Proton radiation therapy has become popular in recent years for many types of cancer, but most doctors and radiologists do not see a benefit in using this treatment for DIPG tumors. Numerous clinical studies have attempted to improve the effectiveness of radiation by combining radiation with radiosensitizers, which are drugs intended to make cancer cells more likely to die from the radiation. None of the studies so far have shown any benefit to adding a radiosensitizer.
In the past 30 years, DIPG patients have participated in more than 250 clinical trials. These trials have involved a wide array of chemotherapy drugs – sometimes alone and sometimes in combination with other drugs. None of these studies have shown any benefit in either the likelihood of survival or the median length of survival. Doctors and researchers are actively searching for effective chemotherapy agents. Given the increased understanding of the biology of DIPG tumors, these new approaches can be tailored to the particular characteristics of the DIPG tumor in ways that prior trials could not. These recent developments have given doctors and researchers reasons to be optimistic that they will develop effective treatments for DIPG in the future.
Medical advances in the past 40 years have greatly improved the survival rates for children diagnosed with most types of cancer. But these medical advances have done nothing for children with DIPG. Brain tumors remain the most common cause of cancer-related death in children, and DIPG is the leading cause of death from pediatric brain tumors. A child diagnosed with DIPG today faces the same prognosis as a child diagnosed 40 years ago. There is still no effective treatment and no chance of survival. Only 10% of children with DIPG survive for 2 years following their diagnosis, and less than 1% survive for 5 years. The median survival time is 9 months from diagnosis.
source: defeatdipg.org
Unlike many brain tumors, a DIPG tumor cannot be removed through surgery. Attempting surgical removal of a brain stem tumor would likely cause severe neurological damage and could even be fatal.Surgery is not an option for two reasons. First, because the pons is located in the center of the brain, a surgeon could not get to the tumor without damaging the surrounding portions of the brain. Second, even if the tumor could be accessed, total removal would be impossible. A DIPG tumor is not a solid, well-defined mass like some tumors. Instead, the tumor cells spread out between the healthy brain cells. Because the tumor cells could not be entirely removed without also removing the healthy cells, even if surgery were performed, the remaining cells would continue to divide and the tumor would soon return.
Radiation is part of the standard course of treatment for DIPG patients, as it is the only form of treatment that has proven benefits. For roughly 70% of DIPG patients, radiation causes the tumor to shrink, which provides relief from many of the symptoms associated with DIPG. The benefits of radiation, however, are only temporary. Even for those patients whose tumors shrink during radiation, the treatment does not increase their chances of survival, and the tumor begins to grow again within months. Because DIPG patients who do not undergo radiation have a median survival length of roughly 5 months, radiation therapy typically extends a patient’s life by about 3 months. Proton radiation therapy has become popular in recent years for many types of cancer, but most doctors and radiologists do not see a benefit in using this treatment for DIPG tumors. Numerous clinical studies have attempted to improve the effectiveness of radiation by combining radiation with radiosensitizers, which are drugs intended to make cancer cells more likely to die from the radiation. None of the studies so far have shown any benefit to adding a radiosensitizer.
In the past 30 years, DIPG patients have participated in more than 250 clinical trials. These trials have involved a wide array of chemotherapy drugs – sometimes alone and sometimes in combination with other drugs. None of these studies have shown any benefit in either the likelihood of survival or the median length of survival. Doctors and researchers are actively searching for effective chemotherapy agents. Given the increased understanding of the biology of DIPG tumors, these new approaches can be tailored to the particular characteristics of the DIPG tumor in ways that prior trials could not. These recent developments have given doctors and researchers reasons to be optimistic that they will develop effective treatments for DIPG in the future.
Medical advances in the past 40 years have greatly improved the survival rates for children diagnosed with most types of cancer. But these medical advances have done nothing for children with DIPG. Brain tumors remain the most common cause of cancer-related death in children, and DIPG is the leading cause of death from pediatric brain tumors. A child diagnosed with DIPG today faces the same prognosis as a child diagnosed 40 years ago. There is still no effective treatment and no chance of survival. Only 10% of children with DIPG survive for 2 years following their diagnosis, and less than 1% survive for 5 years. The median survival time is 9 months from diagnosis.
source: defeatdipg.org
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