Ewing sarcoma family of tumours

Dr Henry Knipe and A.Prof Frank Gaillard et al.

Ewing sarcoma family of tumours (ESFT) are a group of small round blue cell tumours that are closely histogenetically related, all demonstrating non-random t(11;22)(q24;q12) chromosome rearrangement resulting in the formation of the EWS-ETS fusion gene 1-3. They include:

  1. Ewing sarcoma of bone
  2. extraosseous Ewing sarcoma (EES)
  3. peripheral primitive neuroectodermal tumour (pPNET)
  4. Askin tumour
  5. neuroepithelioma

Although the literature is littered with contradictory terminology, all three tumours are likely to represent the same entity with minor differences in differentiation. For example, PNET is more differentiated demonstrating neural elements (e.g. Homer Wright rosettes) 2

As such all three entities should be referred to as Ewing sarcoma, with pPNET / Askin representing extraskeletal Ewing sarcoma.

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Article information

rID: 7854
System: Paediatrics
Section: Gamuts
Synonyms or Alternate Spellings:
  • Ewing sarcoma family of tumours (ESFT)
  • ESFT

Cases and figures

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    Figure 1: histology : small round blue cells
    Drag here to reorder.
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