RESUMO
BACKGROUND: Isolated primary pancreatic lymphoma (PPL) is a rare extra-lymphatic non-Hodgkin's lymphoma comprising less than 1% of all extra-lymphatic lymphomas. It is seen in people of advanced age and there is a slight male preponderance. It is difficult to diagnose; the vague presenting symptoms and nonspecific laboratory/radiological findings make it difficult to differentiate the condition from pancreatic adenocarcinoma. Histopathological examination is of paramount importance to conclusively establish the diagnosis since the treatment involves lymphoma protocols, and prognosis and survival in PPL are considerably superior to that in adenocarcinoma pancreas. CASE REPORT: We report a case of isolated PPL diagnosed after Tru-Cut biopsy and immunohistochemistry after a thorough staging workup. RESULT: The patient was treated with multi-agent combination chemotherapy followed by radiotherapy. DISCUSSION: A review of literature was done using a Medline search to determine the incidence and prevalence of isolated PPL and to note the diagnosis and management of previously reported cases. CONCLUSION: An exceedingly rare entity, isolated PPLs need to be differentiated from pancreatic adenocarcinomas by histopathological evaluation since management is on the lines of other extralymphatic lymphomas and prognosis is significantly better.