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Primitive Neuroectodermal Tumor (PNET) treatment options

Primitive Neuroectodermal Tumor (PNET) treatment options

Question

Dear OncoLink "Ask The Experts,"

My cousin's son, 20 months old was diagnosed with a Primitive Neuroectodermal Tumor (PNET). He has already had surgery. What are the general treatment options for this?  


Answer

Hui-Kuo G. Shu, MD, PhD, an Assistant Professor in the Department of Radiation Oncology at the University of Pennsylvania, responds:

Your question did not specify the location of the tumor. The most common location of a PNET in children is the central nervous system. The standard treatment for a child of this age would consist of chemotherapy following the surgery. Craniospinal irradiation (CSI) is generally deferred until the child reaches an age of 3-4 years so the potential neurocognitive and growth effects are minimized. However, if the child has evidence of progression of the tumor, radiation may be recommended at a younger age. While outcomes for these young patients are not as good as in older patients who receive upfront CSI as part of their therapy, long-term control is achieved in many patients. While newer, more intensive chemotherapy regimens show promising early results, none have been clearly shown to be superior to the standard regimen used for this disease.