Ewing sarcoma/peripheral primitive neuroectodermal tumor and related tumors

Maria Tsokos, Rita D. Alaggio, Louis P. Dehner, Paul S. Dickman

Research output: Contribution to journalReview article

42 Scopus citations

Abstract

Ewing sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and other tumors with EWS gene rearrangements encompass a malignant and intermediate neoplasm with a broad anatomic distribution and a wide age range but a predilection for soft tissue in children, adolescents, and young adults. The overlapping histologic, immunohistochemical and cytogenetic and molecular genetic features create diagnostic challenges despite significant clinical and prognostic differences. Ewing sarcoma is the 3rd most common sarcoma in children and adolescents, and desmoplastic small round cell tumor is a rare neoplasm that occurs more often in older children, adolescents, and young adults. Pathologic examination is complemented by immunohistochemistry, cytogenetics, and molecular genetics. This article reviews the clinicopathologic features of EWS/pPNET and desmoplastic small round cell tumor in the spectrum of tumors with EWS gene rearrangements. Other tumors with different histopathologic features and an EWS gene rearrangement are discussed elsewhere in this volume.

Original languageEnglish
Pages (from-to)108-126
Number of pages19
JournalPediatric and Developmental Pathology
Volume15
Issue numberSUPPL. 1
DOIs
StatePublished - Mar 28 2012

Keywords

  • Desmoplastic small round cell tumor
  • EWS gene rearrangement
  • EWS/pPNET
  • Ewing sarcoma
  • Peripheral primitive neuroectodermal tumor

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