Primary Pancreatic T-Cell Lymphoma - A Case Report and Literature Review

Abstract

Primary pancreatic lymphoma is a rare form of extranodal malignant lymphoma. Most cases show a diffuse large B-cell immunophenotype. Hereby we describe the case of a 62-year-old man that was admitted in the emergency department of Sao Paulo Hospital with abdominal pain, weight loss and jaundice. Imaging exams showed a bulky resectable tumour in the pancreatic head. The patient underwent pancreatoduodenectomy and the histopathological analysis showed an primary pancreatic T-cell lymphoma. He received chemotherapy and subsequent autologous stem-cell transplantation. He presented a complete remission with no evidence of disease in the 8-months follow up. Literature review on this disease recommends the diagnostic to be done with endoscopic or percutaneous biopsy and the best treatment choice is systemic therapy, but evidence is scarce. There are few cases of T-cell primary pancreatic lymphoma described in the literature, with a worse prognosis compared to other immunophenotypes