Ewing sarcoma family of tumors

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

Ewing sarcoma family of tumors are a group of small round blue cell tumors 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

Terminology

Although the literature is littered with contradictory terminology, all three tumors 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.

Pathology

Ewing sarcoma family of tumors include:

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

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

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Cases and figures

  • Figure 1: histology : small round blue cells
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