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About Childhood Primitive Neuroectodermal Tumors (PNETs) and Pineoblastoma

  • Primitive neuroectodermal tumors (PNETs) and pineoblastoma are a group of tumors defined by their appearance. Researchers believe they develop from primitive (undeveloped) nerve cells in the brain. They are similar to medulloblastoma.

    At Dana-Farber/Boston Children's Cancer and Blood Disorders Center, our team of specialized pediatric neuro-oncologists, neuropathologists, and neurosurgeons have expertise in treating rare brain and spine tumors. Children with PNETs or pineoblastoma receive expert care through our Medulloblastoma Program, part of our comprehensive Childhood Brain Tumor Center.

    Symptoms of Childhood PNETs and Pineoblastoma

    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

    How We Diagnose Childhood PNETs and Pineoblastoma

    Your child’s physician may order several different tests to diagnose the tumor. They may include physical and neurological exams and advanced imaging studies.

    After all necessary tests are complete, your child’s medical team will identify the best treatment options. They will meet with you to outline the recommended treatment plan and answer any questions you may have.

    How We Treat Childhood PNETs and Pineoblastoma

    Your child's physician will determine a specific course of treatment based on several factors, including their health status and the tumor’s characteristics.

    Treatments may include:

    • Surgery: We initially use surgery to biopsy the tumor and form a complete diagnosis. Depending on the tumor, we may recommend further surgery to remove as much of the cancer as possible.
    • When a tumor causes blockage of cerebral spinal fluid flow, our surgeons may perform a procedure to relieve symptoms of hydrocephalus, the buildup of fluid inside the skull. In an endoscopic third ventriculostomy (ETV) procedure, surgeons create a small hole that allows fluid to flow around the blockage. We avoid using shunts in children with these conditions.

    • Chemotherapy: Chemotherapy treatment involves medications that interfere with a cancer cell’s ability to grow or reproduce. Doctors use it to shrink tumors and eliminate remaining cancer cells. Different groups of chemotherapy drugs work in different ways. They are generally systemic treatments. Your child may receive chemotherapy in the following ways:
      • Orally, as a pill to swallow
      • Intramuscularly, as an injection into the muscle or under the skin
      • Intravenously (IV), as a direct injection into the bloodstream
      • Intrathecally, as a direct injection into the spinal fluid
    • Radiation therapy: Doctors may use radiation to shrink tumors or damage and destroy cancer cells. Radiation therapy involves using high-energy waves to damage or shrink tumors.

    Beyond initial treatment, we continue to care for the physical, mental, and emotional health needs of children with brain tumors through our extensive pediatric cancer survivorship programs, including the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic for pediatric brain tumor survivors. These services address health and social issues, ranging from motor function evaluation and physical therapy to return-to-school and learning programs.

    Long-term Outcomes for Childrenhood PNETs and Pineoblastoma

    The five-year survival of patients with PNETs or pineoblastoma is 50 to 60 percent. The outcomes are less favorable in infants and children who have partial surgical removal and an inadequate response to radiation therapy.

    Childhood PNETs and Pineoblastoma Treatment Team

    Our treatment team includes specialists dedicated to treating children with PNETs and pineoblastoma. See a complete list of the specialists in our Childhood Brain Tumor Center.