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Definition

A highly malignant, metastatic, primitive small round cell tumor of bone and soft tissue that affects children and adolescents. It belongs to the Ewing sarcoma family of tumors, a group of morphologically heterogeneous neoplasms that share the same cytogenetic features. They are considered neural tumors derived from cells of the neural crest. Ewing sarcoma represents the less differentiated form of the tumors.

Acronym

ES

Synonyms

Askin tumor
ESFT
Ewing's tumor
Ewing sarcoma family of tumors
Extraosseous Ewing tumor
Peripheral neuroepithelioma
PNE
PNET
PNET of the chest wall
Primitive neuroectodermal tumor

 
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