What is the prevalence of primitive neuroectodermal tumors (PNETs)?

Updated: Feb 07, 2019
  • Author: Carlo P Honrado, MD, FACS; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Answer

Answer

The incidence of peripheral primitive neuroectodermal tumors (pPNETs) is likely underreported in the literature because recent diagnostic advances have allowed these tumors to be distinguished from other small, poorly differentiated, round cell tumors. Although peripheral primitive neuroectodermal tumors (pPNETs) are exceedingly rare, the annual incidence of tumors from the larger Ewing family of tumors (EFTs) from birth to age 20 years is 2.9 per million population. In most large series published to date, peripheral primitive neuroectodermal tumors (pPNETs) usually present in the second decade of life, with a slight male preponderance. They account for 4-17% of all pediatric soft tissue tumors. [9] Of note, these tumors are rare in African American children and children of Asian descent, with most worldwide cases occurring in white and Hispanic children and adolescents.


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