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What are the treatments for PNET?
Specific treatment for PNET will be determined by your child's physician based on:
Treatment may include (alone or in combination):
If there is hydrocephalus (swelling of the brain) in a pineoblastoma, another procedure called an ETV (endoscopic third ventriculostomy) may be necessary. There have been reports of these tumors traveling through shunts into the peritoneal cavity; therefore, all efforts are made to avoid placement of a shunt in these children.
How is chemotherapy given?
Different chemotherapies may be given:
How is chemotherapy used?
This depends on many factors. Some things to keep in mind:
Does chemotherapy come with bad side effects?
While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help your child, family and your child's health care team prepare for and sometimes prevent these symptoms from occurring.
How are side effects managed?
The side effects of therapy will depend on the surgery and other treatments that have been performed, including radiation therapy to the brain and spine.
Treatment for these side effects may include physical and occupational therapy (if your child experiences weakness or sensory loss), and speech therapy (if her speech is impaired), all on either an inpatient or outpatient basis.
What is the expected outcome after treatment for PNET?
Since these tumors are so rare and hard to classify, it's difficult to analyze outcome and survival rates. The overall 5-year survival of patients with PNET is between 50 and 60 percent, but is clearly worse among infants and pineoblastoma patients with incomplete tumor removal or poor response to radiation therapy.
What about progressive or recurrent disease?
Relapsed PNET is almost always fatal and there are no good chemotherapy or other curative therapies available. There are trials looking at bone marrow transplantation with aggressive chemotherapy and use of novel biologic agents, but most of these are currently experimental.
What is the latest research on PNET?
A variety of chemotherapeutic regimens have been evaluated in the treatment of newly diagnosed high-grade gliomas.
How Dana-Farber/Boston Children's approaches PNET
Children with PNET are treated through the Brain Tumor Program at Dana-Farber/Boston Children's Cancer and Blood Disorders Center, a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors. Boston Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help clinicians better understand and treat PNET.
We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”