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Primitive Neuroectodermal Tumors Defined
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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