Primary pancreatic lymphoma (PPL) is a rare tumor that mimics pancreatic adenocarcinoma, leading to diagnostic and therapeutic challenges. PPL accounts for 0.2% of all pancreatic tumors and is typically treated with chemotherapy. However, the long-term survival rates for PPL with chemotherapy and radiotherapy alone are unsatisfactory. Due to the improvements in pancreatic surgery, there is a need to reevaluate the treatment strategies for PPL.

A 62-year-old male presented to our clinic. A biopsy was unsuccessful, and the imaging was suggestive of pancreatic adenocarcinoma. Therefore, subtotal splenopancreatectomy was performed and histopathology was performed. He was then diagnosed with primary pancreatic diffuse large B-cell lymphoma. He received adjuvant chemotherapy and radiotherapy. Currently, the patient is alive with no evidence of disease 36 months after surgery.

The potential role of surgery in the treatment of PPL should be emphasized and added in the management protocol of early stage lymphoma.

Primary lymphomas of endocrine glands are extremely rare. Our study adds more data to the few published series regarding the incidence, clinical characteristics, management and overall survival (OS) by comparing the various diffuse large B-cell endocrine lymphomas. Moreover, it contributes to a better understanding of these neoplasms and provides concepts for future research.

We retrospectively evaluated the clinical profile and the patterns of outcome among patients who were treated in our center with the diagnosis of aggressive, B-cell, primary endocrine lymphoma.

Between May 1980 and December 2011, 450 patients were diagnosed as primary extranodal non-Hodgkin lymphomas. Among them, 18 cases (4%) were primary testicular lymphoma (PTL), 8 cases (2%) were primary thyroid lymphoma (PTHL) and 4 cases (1%) were primary adrenal lymphoma (PAL). The therapeutic approaches employed were variable, including mainly chemotherapy in combination with radiotherapy and surgery. The median OS for the patients with PTL and PAL was 27 and 6 months, respectively. Better outcome was observed in patients with PTHL for whom the median OS has not been reached yet, whereas the PAL group had the worst prognosis.

The discrepancies in the outcome among endocrine lymphomas could be partly attributed to their biologic variability, which might be determined by the initial site involved. We conclude that treatment decisions should be made according to a multi-disciplinary approach to avoid unnecessary surgery. Existing treatment strategies for PTL and PAL fail to provide long-term survival, rendering the application of novel therapeutic approaches essential.

Hematologic malignancies often involve the pancreas, causing potential diagnostic pitfalls and, rarely, potentially avoidable surgical resection. We review the spectrum of hematologic malignancies involving the pancreas and describe features useful in preoperative distinction from adenocarcinoma. Archived clinical, pathologic, and radiologic data (1965 to present) for hematologic malignancies involving the pancreas were reviewed and compared with the data for 157 surgically resected pancreatic adenocarcinomas. Of 42 cases, 27 (64%) were clinically "suspicious" for hematologic malignancies. Of the remaining 15 cases, 4 patients underwent resection for presumed pancreatic adenocarcinoma. Isolated pancreatic masses proved most difficult to identify clinically. Significant factors in distinguishing hematologic malignancies from adenocarcinoma included history of hematologic malignancy, young age, large tumor size, low CA19-9 level, B symptoms, and lack of jaundice or diabetes mellitus. Various hematologic malignancies involve the pancreas, most commonly diffuse large B-cell lymphoma. Pancreatic masses are usually correctly identified clinically. Preoperative and operative sampling is strongly recommended when hematologic malignancies cannot be excluded.

Pancreatic masses can be solid or cystic, benign or malignant. Rapid and accurate diagnosis is essential for optimal management. Clinical presentation and radiological appearance are often inadequate for a definitive diagnosis. Endoscopic ultrasound allows more detailed assessment of the pancreas than traditional imaging techniques.