Background: Extraosseous Ewing's sarcoma (EES) is a mesenchyme-derived small blue cell tumor, which is distinguished by its rarity, aggressiveness, dismal prognosis, and distinct pathogenesis. Occurring almost exclusively among children and young adults, EES can arise from a variety of organs and portends a rapid clinical deterioration and high likelihood of recurrence.
Discussion: We present the first reported case of a primary pancreatic Ewing's sarcoma in a patient with concomitant portal vein thrombosis. The atypical presentation of this extraordinarily rare tumor underscores the imperative to maintain EES in the differential diagnosis of suspicious, indistinct pancreatic lesions in young patients. In addition, we review the available literature describing additional cases of primary pancreatic Ewing's sarcoma.