Abstract

Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a family of very similar “small round blue cell” tumors that typically occur in the bone or soft tissue of children and young adults. Although historically these tumors were often subdivided into distinct diagnostic entities on the basis of clinical and histological variations, they are now all regarded as a single entity in light of molecular and immunohistochemical findings. ES/PNET occurring as a primary skin tumor is rare with fewer than 50 cases reported in the literature mainly as case reports and several small case series.

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