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Primitive Neuroectodermal Tumors Defined

Originally published on January 22, 2013
Most recently updated on April 10, 2013

Primitive neuroectodermal tumors, or PNETs, account for approximately 5% of pediatric brain tumors. They are a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissue and bone


    •    CNS primitive neuroectodermal tumors (PNETs) - Tumors derived from the central nervous system
    •    Neuroblastoma - Tumors derived from the autonomic nervous system
    •    Peripheral primitive neuroectodermal tumors (pPNETs) - Tumors derived from tissues outside the central   and autonomic nervous system


Symptoms depend of their location and the proximity to cerebral spinal fluid (CSF) spaces.


Treatment options include: surgical resection, radiation therapy to the brain and spine, and chemotherapy.


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