Objective   To investigate the clinical characteristics, diagnosis, and treatment of primary pancreatic lymphoma (PPL).

  Methods   Clinical data of six PPL cases diagnosed and treated in the Tianjin Medical University Cancer Hospital were retrospectively analyzed.

  Results   In all cases, the primary clinical manifestations were abdominal pain, jaundice, and weight loss. The tumor marker level, including CA19-9, of all the cases was within the normal range, with five of the cases misdiagnosed as pancreatic cancer and one misdiagnosed as stomach cancer. In three cases, the pancreatic mass was located at the head of the pancreas, whereas in the other three, the mass was located at the body or tail. Pancreaticoduodenectomy was conducted in one case, laparotomy and biopsy in three cases, and B ultrasound-guided biopsy in the other two cases. All cases were diagnosed as PLL (B cell type non-Hodgkin's lymphoma). Except for one patient who left the hospital after surgery, the other five underwent chemotherapy. In addition to the death in one case because of postoperative gastrointestinal bleeding, the survival times were 133, 73, 35, 12, and 10 months in the remaining cases.

  Results   Conclusion: PPL is extremely rare in clinical practice, and the treatment methods and prognosis for the disease significantly differ from those for pancreatic cancer. PPL is easily misdiagnosed in clinical practice, which results in unnecessary treatment measures.