• Primitive Neuroectodermal Tumors (PNET) and Pineoblastoma

    Primitive Neuroectodermal Tumors (PNET) and pineoblastoma are a group of tumors defined by their appearance and are thought to develop from primitive (undeveloped) nerve cells in the brain. They appear similar to medulloblastoma and were once considered a single tumor. 

    PNET Treatment at Dana-Farber/Boston Children's

    Patients with PNETs and pineoblastoma are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Medulloblastoma Program. Continue reading to learn more about PNETs/pineoblastomas or visit the Medulloblastoma Program homepage to learn about our expertise and approach to treating this condition.

    Symptoms & Diagnosis

    PNETs and pineoblastomas are aggressive tumors that tend to attach to parts of the brain that control movement, thought and sensation. Scientists have not been able to find an identifiable cause or risk factors for these tumors. There doesn't appear to be a genetic predisposition, meaning that these diseases do not seem to run in families.

    Symptoms depend on the location of the tumor, and each child may experience symptoms differently. Common symptoms include headache, nausea and vomiting, fatigue, lethargy, seizures, behavior or personality changes, unexplained weight loss or gain, difficulty looking upward and weakness on one side of the body.

    Your child’s physician may order a number of different tests to best diagnose the tumor. In addition to a physical exam, medical history and neurological exam (which tests reflexes, muscle strength, eye and mouth movement, coordination and alertness), your child’s doctor may requests tests, including diagnostic imaging.

    Since these tumors are known to spread via cerebrospinal fluid, there is a high chance that they will invade other tissues of the brain and spine, so it is essential that your child have an MRI of both the brain and spine.

    After all necessary tests are complete, the best treatment options can be identified.

    Treatment & Care Options

    Your child’s physician will determine a specific course of treatment based on several factors. Some therapies will treat the tumor while others are intended to address complications of the disease or side effects of the treatment. These treatments include:

    If your child has hydrocephalus (swelling of the brain), another procedure, called 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 shunt placement in these children.

    There can be side effects related to the tumor itself or its treatment. Knowing what these side effects are can help your care team prepare for and, in some cases, prevent these symptoms from occurring.

    Progressive or Recurrent Disease

    Recurrent PNET or pineoblastoma is almost always fatal, and there are no good chemotherapy or other curative treatments 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.

    Long-term Outlook

    Overall, the five-year survival of patients with a PNET or pineoblastoma is 50 percent to 60 percent, but it is clearly worse among infants or in patients with incomplete surgical removal and poor response to radiation therapy.
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