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Treatment Options for Newly Diagnosed CNS Primitive Neuroectodermal Tumors

    Children Older Than 3 Years

    Standard treatment options

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    Attempting aggressive surgical resection is the first step in the management of newly diagnosed central nervous system primitive neuroectodermal tumors (CNS PNETs), although studies have yet to demonstrate that the extent of resection is predictive of outcome.[1,2,3] After surgery, children with CNS PNETs usually receive treatment similar to that received by children with poor-risk medulloblastoma. Best results have been obtained after radiation to the entire neuraxis with local boost radiation therapy, as given for medulloblastoma.[3] However, the local boost radiation therapy may be problematic because of the size of the tumor and its location in the cerebral cortex. The chemotherapeutic approaches during and after radiation therapy are similar to those used for children with poor-risk medulloblastoma. Three- to 5-year overall survival (OS) rates of 40% to 50% have been noted.[1,2,3]; [4][Level of evidence: 2A]; [5][Level of evidence: 3iiiB] Patients with disseminated disease at the time of diagnosis have poorer OS, with reported survival rates at 5 years ranging from 20% to 30%.[1,2,3]

    Children Aged 3 Years and Younger

    Standard treatment options

    Treatment of children aged younger than 3 years with CNS PNETs is similar to that outlined for children with medulloblastoma. With the use of chemotherapy alone, outcome has been variable, with survival rates at 5 years ranging between 0% and 50%.[6,7,8]; [9][Level of evidence: 2Di]

    Treatment options under clinical evaluation

    The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.

    • COG-ACNS0334(Combination Chemotherapy Followed By Peripheral Stem Cell Transplant in Treating Young Patients With Newly Diagnosed Supratentorial PNETs or High-Risk Medulloblastoma): This Children's Oncology Group phase III randomized trial is studying children aged 3 years and younger with high-risk medulloblastoma or CNS PNETs. Patients are randomly assigned to intensive induction chemotherapy including or excluding methotrexate followed by consolidation with hematopoietic stem cell rescue.

    Current Clinical Trials

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