Primitive Neuroectodermal Tumor (PNET) and Extraskeletal Ewing’s Sarcoma

  • Mario Campanacci


PNET is a tumor composed of primitive neuroblasts (similarly to a neuroblastoma), but not arising in relationship to the sympathetic ganglia or the adrenal glands. Thus it is also named peripheral neuroblastoma, or peripheral neuroepithelioma. Extraskeletal Ewing’s sarcoma is histologically identical to Ewing’s sarcoma of bone, and there is evidence that it represents the less differentiated end of the spectrum of PNET. Askin’s tumor is a small round cell sarcoma occurring in the thoracic wall (soft tissues or rib) of children and again it represents a clinical variety of PNET.


Primitive NEUROECTODERMAL Tumor Small Round Cell Tumor Dense Core Granule Intergroup Rhabdomyosarcoma Study Peripheral Neuroectodermal Tumor 
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Copyright information

© Springer-Verlag Wien 1999

Authors and Affiliations

  • Mario Campanacci
    • 1
    • 2
  1. 1.University of BolognaItaly
  2. 2.Rizzoli Orthopaedic InstituteBolognaItaly

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