Primary Ewing sarcoma of the pancreas: A rare extraosseous presentation

Ewing sarcoma is an aggressive neoplasm that is common among children and adolescents, most frequently affecting the pelvis and long bones. Although rare, Ewing sarcoma is known to also occur in extraosseous locations, affecting a broad range of soft tissue structures. The diagnosis of extraosseous Ewing sarcoma is difficult given the nonspecific symptomatology, often related to local tumor burden. Radiological features of this entity overlap with other common malignancies, as Ewing sarcoma typically presents on computed tomography (CT) as a heterogeneous mass containing areas of necrosis and calcification. Imaging plays a central role in the evaluation of this entity, but histologic and molecular analysis remain necessary for definitive diagnosis. We report the case of a 40-year-old female with a primary pancreatic extraosseous Ewing sarcoma. We correlate clinical indicators with imaging findings and pathological analysis to discuss this patient’s long-term outcome following multimodal therapy.