Primary pancreatic follicle center-derived lymphoma masquerading as carcinoma.

Abstract

Primary pancreatic lymphoma is a rare neoplasm that clinically mimics pancreatic and periampullary carcinoma. This condition comprises a heterogeneous group of lymphoproliferative disorders, of which diffuse large B-cell lymphoma is the most common. The clinical, radiologic, and laboratory features of primary pancreatic lymphoma often overlap with those of carcinoma, but primary pancreatic lymphoma typically presents as a bulky tumor and lacks the features typically associated with pancreatic carcinoma, such as referable back pain, significant pancreatic and/or bile duct obstruction or alteration, obstructive jaundice, and peripancreatic vascular occlusion or thrombosis. Additional findings that favor a diagnosis of lymphoma include regional lymphadenopathy, particularly infrarenal nodes, and markedly elevated levels of sIL-2R. The clinicopathologic diagnosis and classification of primary pancreatic lymphoma are critical for appropriate treatment and clinical management; adequate tumor sampling for pathologic and flow cytometric evaluation can be obtained by imaging-guided, endoscopic, or surgical means. The treatment of primary pancreatic lymphoma consists of aggressive combination chemotherapy. Following the addition of rituximab to standard chemotherapeutic regimens, the role of surgery with curative and palliative intent needs further evaluation.