What is primitive neuroectodermal tumor of the thoracopulmonary region (PNET)?

Updated: Dec 25, 2019
  • Author: Joseph F Tomashefski, Jr, MD; more...
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Answer

Answer

The PNET is a member of the Ewing sarcoma (EWS) family of tumors and bears a close resemblance to, if not identity with, EWS. [16, 17, 18] PNET typically occurs in children and young adults. The frequent occurrence of extraosseous PNETs in the thoracopulmonary region was first described by Askin and colleagues in 1979. [19] The Askin tumor, like other PNETs, is composed of small round cells with a high nuclear-to-cytoplasmic ratio. It is primarily a pleural-based tumor that may extend into the soft tissue of the chest wall or the ipsilateral lung.

The distinction between PNET and EWS is at times difficult, if not impossible, to discern. Therefore, tumors in this spectrum are often referred to as the combined designation PNET/EWS.


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