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Ewing sarcoma family of tumors

Last revised by Joshua Yap on 26 Apr 2023

The 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


Although the literature is littered with contradictory terminology, all of these tumors are likely to represent the same entity with minor differences in differentiation. For example, pPNET is more differentiated as it demonstrates neural elements (e.g. Homer Wright rosettes) 2. As such, all of these entities should be referred to as being in the Ewing sarcoma family of tumors.


The Ewing sarcoma family of tumors include:

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Cases and figures

  • Figure 1: histology (small round blue cells)
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  • Case 1: Ewing sarcoma of the rib
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  • Case 2: Ewing sarcoma of the chest wall
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