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.
Terminology
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.
Pathology
The Ewing sarcoma family of tumors include:
extraskeletal Ewing sarcoma (EES): a literal descriptor, which includes the below conditions (apart from the osseous Ewing sarcomas of the chest wall) 4
Ewing sarcoma of the chest wall: includes tumors that fell under the original description of Askin tumor (as arising from the soft tissues of the chest wall, as well as from osseous structures including the rib, scapula, clavicle, or sternum)
primitive neuroectodermal tumors (PNETs, formerly known as peripheral-neuroepitheliomas): an umbrella term that includes peripheral primitive neuroectodermal tumor (pPNET), PNET of pancreas, and PNET of kidney 5
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