Primary Extraskeletal Ewing Sarcoma of the Pancreas - A Case Report

Abstract

Extraskeletal Ewing sarcomas (EES) is a rare, aggressive malignancy that typically affects adolescents or young adults, primarily involving the deep soft tissues of the lower extremities and paravertebral regions. The occurrence of EES in the pancreas is even rarer. These tumors are characterized by small, round cell sarcomas displaying varying degrees of neuroectodermal differentiation, as revealed through light, electron microscopy, or immunohistochemistry. Diagnosing EES demands a high level of suspicion. Histopathologically, the presence of small round cell tumors in the pancreas, along with CD99 positivity in immunohistochemistry, assists in diagnosing EES. Molecular analysis demonstrating EWSR1 (22q12) rearrangement via interface fluorescence in situ hybridization is required to confirm the diagnosis. A comprehensive review of pancreatic EES cases revealed that the primary treatment modality typically involves surgical intervention, often complemented by chemotherapy and, in some cases, radiotherapy. In this report, we describe the case of a 28-year-old male presenting with abdominal pain and a loss of appetite, which, upon histopathological and molecular examination, was identified as EES of the pancreas. The patient underwent surgical resection of the pancreatic mass, followed by omentum, splenectomy, and chemotherapy. EES is a highly aggressive tumor with an insidious onset, and patients usually exhibit non-specific clinical symptoms. Although exceedingly rare, it should be considered in the differential diagnosis of pancreatic masses.

Keywords

• extraskeletal
• sarcoma
• ewing
• pancreas
• case report