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A 48-year-old man with cirrhosis secondary to chronic hepatitis C and alcoholic liver disease presented with abdominal pain, hematemesis, and melena. Physical examination showed that the patient had jaundice with epigastric tenderness. Laboratory results yielded the following values: total bilirubin, 11 mg/dL; direct bilirubin, 8.5 mg/dL; alkaline phosphatase, 164 U/L; aspartate aminotransferase, 99 U/L; alanine aminotransferase, 72 U/L; and carbohydrate antigen 19-9, 86 U/mL. Computed tomography of the abdomen showed a large mass involving the head of the pancreas. Magnetic resonance cholangiopancreatography revealed a large heterogeneous mass arising from the pancreatic head and compressing the common bile duct. Endoscopic retrograde cholangiopancreatography showed a large ulcerative mass in the second part of the duodenum. Cholangiography revealed narrowing of the common bile duct and common hepatic duct. Symptoms, imaging studies, and tumor markers mimicked pancreatic adenocarcinoma, but duodenal mucosa biopsies showed diffuse large B-cell lymphoma. Computed tomography—guided biopsy of the pancreatic mass (hematoxylin-eosin, original magnification, ×20) revealed the same pathologic findings.
Primary pancreatic lymphoma is a rare nonepithelial tumor that arises from the lymphatic tissue of the pancreas. These lymphomas constitute less than 1% of all extralymphatic lymphomas and 0.7% of all pancreatic malignancies.1 Most patients present with nonspecific and vague abdominal symptoms.2 Because of difficulty distinguishing primary pancreatic lymphoma from pancreatic adenocarcinoma, biopsy of all pancreatic masses is essential to establish a definitive diagnosis and exclude potentially curable conditions like primary pancreatic lymphoma.3 Treatment is mainly with chemotherapy (rituximab, cyclophosphamide, hydroxydaunomycin [doxorubicin], vincristine [Oncovin], prednisone [R-CHOP]), with or without adjuvant radiotherapy.2