How are primitive neuroectodermal tumors (PNETs) characterized?

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

Answer

Primitive neuroectodermal tumors (PNETs) are a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissue and bone. Primitive neuroectodermal tumors (PNETs) exhibit great diversity in their clinical manifestations and pathologic similarities with other small round cell tumors. This has made classifying this family of tumors challenging and controversial. Batsakis et al (1996) divided the primitive neuroectodermal tumor (PNET) family of tumors into the following three groups based on the tissue of origin [1] :

  • Central nervous system (CNS) primitive neuroectodermal tumors (PNETs) - Tumors derived from the CNS [2]

  • Neuroblastoma - Tumors derived from the autonomic nervous system

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

Peripheral primitive neuroectodermal tumors (pPNETS) are the focus of this review. See the image below.

Photomicrograph shows characteristic cytoplasmic C Photomicrograph shows characteristic cytoplasmic CD-99 staining in peripheral primitive neuroectodermal tumor.

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