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.

Pancreatic B-cell lymphoma is rare; it accounts for 0.2% to 2.0% of extranodal non-Hodgkin lymphoma, and constitutes less than 0.5% of all pancreatic malignancies. Most histologic types of the pancreatic lymphoma are diffuse large B-cell lymphoma, and follicular lymphoma is quite rare. We report here a case of pancreatic follicular lymphoma that was initially detected by acute pancreatitis. This is the first reported case of pancreatic follicular lymphoma presenting with acute pancreatitis. A 71-year-old woman had epigastric and left upper quadrant abdominal pain. Computed tomography (CT) revealed features of acute pancreatitis. After standard therapy for pancreatitis, enhanced CT showed a pancreatic tumor (50 × 35 mm) in the body of the pancreas with gradual enhancement. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography showed a complete interruption of the pancreatic duct in the body, with mild dilation of the duct in the tail of the pancreas. Endoscopic ultrasonography revealed hypervascularity of the pancreatic tumor. The patient underwent distal pancreatectomy to remove the cause of pancreatitis and to disclose the diagnosis. Histologic examination revealed follicular lymphoma of pancreas. Despite recent improvement in clinical strategies, differential diagnosis between pancreatic lymphoma and pancreatic cancer is still difficult without histologic information. Pancreatic lymphoma should be considered as a differential diagnosis in a patient who initially presents with acute pancreatitis.

A 69 year old man with chief complaints of vomiting and nausea was referred to our hospital. Ultrasonographic examination on 19 May 1998 showed a hypoechoic homogeneous mass 6 cm in diameter in the head of the pancreas. Degree of intravascular infiltration was angiographically mild despite its large size. Tumor-marker levels were normal. On 9 August 1998, however, ultrasonography showed the mass to have rapidly grown (10 cm in diameter) including a heterogeneous internal structure. The mass had a linear or branching signal pattern.

To describe the imaging findings of primary and secondary pancreatic malignant lymphoma on magnetic resonance imaging (MRI), to help differentiate lymphoma of the pancreas from primary adenocarcinoma and autoimmune pancreatitis among others, and to discuss a few atypical presentations of pancreatitis mimicking lymphoma.

Knowledge of these imaging manifestations of lymphoma may be helpful to arrive at an accurate diagnosis and avoid unnecessary morbidity and mortality from inadvertent surgery.

• Pancreatic malignant lymphoma is shown as a nodular low-density area with mild enhancement on CT. • It sometimes shows variable manifestations mimicking other tumours and inflammatory conditions. • MRI provides useful information for differentiating malignant lymphoma from other mimickers.

Non-Hodgkin lymphoma predominantly involving the pancreas is a rare tumor and accounts for less than 0.7% of all pancreatic malignancies and 1% of extranodal lymphomas. Diagnosis of primary pancreatic lymphoma can be difficult because it may mimic carcinoma. The principal aims of this review were to highlight the difficulties encountered in making a diagnosis and to identify the role of surgery.

A PubMed search was conducted using the following terms: primary pancreatic lymphoma and non-Hodgkin lymphoma of the pancreas. Additional references were sourced from key articles.

A total of 89 reported cases of pancreatic lymphoma between 1951 and 2005 were reviewed. An accurate preoperative diagnosis of primary pancreatic lymphoma is not always possible. A complete response rate of 100% and a long-term survival rate of 94% have been reported with surgery and adjuvant chemotherapy when compared with a 5-year survival rate of less than 50% and an overall 3-year disease-free survival rate of 44% with current chemotherapy, radiotherapy, or combined methods.

Pancreaticoduodenectomy may have a therapeutic role in association with chemotherapy.

We report the magnetic resonance (MR) appearance of a large B-cell lymphoma in the peripancreatic head region, in a 38-year-old male who presented with a 1-month history of pruritus and jaundice. Routine laboratory examination at presentation revealed an elevated bilirubin. The tumor was a large, solitary well-defined mass with no evidence of necrosis, which showed mild diffuse heterogeneous enhancement. The tumor was closely applied to the lateral margin of the head of the pancreas. The constellation of MR findings was interpreted as consistent with the correct eventual diagnosis of lymphoma.

Pancreatic lymphoma presenting with clinical diabetes mellitus (DM) is rare. We report angiocentric T-cell lymphoma of the pancreas in a 65-year-old Thai woman who presented with progressive deterioration of visual acuity of both eyes. She had diabetic retinopathy (DR) diagnosed only 20 months after the diagnosis of DM at the age of 63. She later developed erythematous rashes, fever, and deterioration of consciousness; she eventually died of shock. A skin biopsy and bone marrow examination revealed angiocentric T-cell lymphoma. At autopsy, the pancreas and both eyes were extensively infiltrated by lymphoma. Widespread involvement of nearly all organs but superficial lymphadenopathy was detected. In contrast to other typical cases of long-standing DM, only mild atherosclerosis was noted, and no DR was found. To the best of our knowledge, this is the first case of lymphoma involving the pancreas and both eyes producing clinical DM and DR.

Pancreatic lymphoma is a rare neoplasm. The role of surgical resection in curing this disease is poorly defined.

From March 1983 to July 1997, eight patients with stage I or II primary pancreatic lymphoma were identified and retrospectively reviewed. All patients received chemotherapy, five patients received radiotherapy, and three patients also underwent surgical resection. A review of the published pancreatic lymphoma experience in the English-language literature was also undertaken.

Three patients underwent pancreaticoduodenectomy with successful resection of the lymphoma and are disease free at 64, 62, and 53 months followup. Five patients were treated with nonresectional therapy. Three are disease free at 128, 51, and 24 months. Two patients died of disease at 9 and 37 months. A review of the pancreatic lymphoma experience in the English-language literature identified 122 cases of pancreatic lymphoma. Fifty-eight of these cases represented stage I or II lymphoma, which was treated without surgical resection with a 46% cure rate. Fifteen patients who had surgical resection for localized disease have been reported with a 94% cure rate.

Based on both our single institution experience and the literature, it is suggested that surgical resection may play a beneficial role in the treatment of localized pancreatic lymphoma, although selection factors cannot be absolutely excluded.