Virginia Commonwealth University Richmond, Virginia
Taseen A. Syed, MBBS, MD1, Robert Lippman, MD2, Tilak Shah, MD, MHS3; 1Virginia Commonwealth University, Richmond, VA; 2Hunter Holmes McGuire VA Medical Center, Richmond, VA; 3Central Virginia VA Healthcare System and Virginia Commonwealth University, Richmond, VA
Introduction: Ewing’s sarcoma (ES) typically occurs as a lytic tumor of the bones. Extra-osseous ES of the pancreas is an extremely rare entity. We report an interesting case of primary pancreatic ES presenting as pancreatic mass with obstructive jaundice.
Methods: 23-year-old Caucasian male presented with 4 weeks of intermittent epigastric discomfort. He denied fever or weight loss. He denied tobacco or heavy alcohol use, and any family history of gastrointestinal malignancies. On physical examination, there was tenderness to palpation of the epigastrium. Laboratory studies were pertinent for anemia (hemoglobin 12.9 mg/dL) and abnormal liver enzymes (total bilirubin 6.8 mg/dL, direct bilirubin 3.3 mg/dL, alkaline phosphatase 121 IU/L, aspartate transaminase 168 IU/L, and alanine transaminase 335 IU/L). Computerized Tomography (CT) of the abdomen demonstrated a 4.7 x 4.9 cm mixed density mass with ill-defined margins in the pancreatic head (Figure A, arrow) with increased uptake on Positron Emission Tomography (PET) scan. Fine needle aspiration demonstrated small round blue cells (Figure B). The slides stained positive for CD56 and negative for synpatophysin, neuron-specific enolase (NSE), anti-pan cytokeratin antibody (AE1/AE3), and CD45 that supported a diagnosis of ES (Figure C). The patient underwent biliary stenting to relieve obstructive jaundice, and was treated with standard chemotherapeutic regimen for this malignancy which includes vincristine, doxorubicin, and cyclophosphamide along with ifosfamide and etoposide (VDC/IE). The pancreatic mass decreased in size over the next few months, and obstructive jaundice resolved. Discussion: Originally reported in 1921, ES typically occurs as a lytic tumor of the bones. Extraosseous ES of the pancreas is an extremely rare entity; in fact, only 24 such cases have been reported previously. Unlike other pancreatic malignancies (e.g. adenocarcinoma, neuroendocrine tumors, or lymphoma), pancreatic ES exhibits similar attenuation to that of muscle on CT and MRI imaging. As was the case in this patient, PET scan typically exhibits increase radionuclide uptake.
Figure A: Computerized Tomography (CT) of the abdomen demonstrate a 4.7 x 4.9 cm mixed density mass with ill-defined margins in the pancreatic head that was diagnosed as Ewing Sarcoma on fine needle aspiration (FNA) analysis.
Figure B: Fine needle aspiration (FNA) histology analysis demonstrating small round blue cells diagnostic for Ewing Sarcoma.
Figure C: CD56 positive slides that were negative for synpatophysin, neuron-specific enolase (NSE), anti-pan cytokeratin antibody (AE1/AE3), and CD45 supporting a diagnosis of Ewing Sarcoma.
Disclosures: Taseen Syed indicated no relevant financial relationships. Robert Lippman indicated no relevant financial relationships. Tilak Shah indicated no relevant financial relationships.