Primary Pancreatic Anaplastic Large Cell Lymphoma, ALK-: A Care Report.

INTRODUCTION: Primary pancreatic lymphomas constitute less than 0.5% of pancreatic malignancies and less than 2% of extranodal lymphomas. Primary pancreatic anaplastic large cell lymphomas (PPALCL) are extremely rare.

AIMS-METHODS: To present an extremely rare case of PPALCL

RESULTS: An 80-year-old man, who was under evaluation of a fever of unknown origin in a Private Health Center, was admitted to our Clinic for further investigation. He noted anorexia, weight loss and fatigue. On physical examination, he had a leg edema. His blood tests were unremarkable except for normochrome normocyte anaemia (Ht 31%, Hb 9,9 g/dl) and a great elevation of ESR (110 mm), LDH (1880 U/L)and β₂-microglobulin (4,19 mg/L). In CT and MRI scan, available from patient’s file, a soft tissue mass located in the pancreas, especially the uncinate process and a slow signal flow of inferior vena cava, due to mass compression, had been observed. He had undergone a normal gastrointestinal endoscopy at that time whereas a new endoscopy, after his admission to us, revealed an ulcerated mass-like deformity of the duodenal bulb with a friable surface. Explorative laparotomy confirmed a diffuse spread of an unresectable malignant mass of the pancreas extending to the adjacent organs. Biopsy specimen was taken from the adhesion of pancreatic surface and gastric serosa, because of the brittle consistency and and necrotic surface of the mass. The duodenal and surgical biopsy results identified an ALCL of T-cell lineage, ALK negative. Bone marrow biopsy showed no evidence of lymphoma involvement. Chemotherapy treatment was not initiated because he died in Intensive Care Unit two days after the explorative laparotomy, due to hemodynamic instability.

CONCLUSIONS: Our case supports that primary pancreatic lymphoma should be a high diagnostic possibility in a patient with a pancreatic mass accompanied by elevated LDH and β₂-microglobulin. Biopsy specimens’ results, from the mass or the adjacent infiltrated organs, confirm the diagnosis, identify the lymphoma and lead to an appropriate and timeable treatment.