Primary pancreatic lymphomas

Abstract

Primary pancreatic lymphomas are extremely rare. Clinically, primary pancreatic lymphomas usually present with symptoms of carcinoma of the pancreatic head. Patients with primary pancreatic lymphomas are between 35 and 75 years of age and with a strong male predominance. Common clinical manifestations include abdominal pain, jaundice, acute pancreatitis, small bowel obstruction, and diarrhea. An accurate cytopathologic diagnosis by fine-needle aspiration (FNA) is imperative because the primary treatment is non-surgical. Cytomorphologic features include hypercellularity with discohesive cells with round nuclei, often prominent nucleoli, mitoses, and karyorrhexis. Flow cytometry analysis demonstrates a monoclonal pattern of immunoglobulin light chain expression. FNA coupled with flow cytometry analysis appears to be highly accurate in the diagnosis of primary pancreatic lymphomas. Fluorescence in-situ hybridisation technique has been established its role in the diagnosis of lymphoid malignancies, including primary pancreatic lymphomas. LDH and beta-2 microglobulin are important diagnostic and prognostic tumor markers. The differential diagnoses of primary pancreatic lymphomas include secondary lymphoma, pancreatic endocrine neoplasm, and florid chronic pancreatitis. The role of surgery is limited to the rare occasions when initial FNA and flow cytometry analysis are non-diagnostic. Treatment usually consists of a combination of chemotherapy and radiation therapy, or stem cell transplantation. Primary pancreatic lymphomas has a much better prognosis than adenocarcinoma of the pancreas.