Primary Pancreatic Lymphoma Research Articles

MR features of primary and secondary malignant lymphoma of the pancreas: a pictorial review

ObjectiveTo 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.ConclusionKnowledge of these imaging manifestations of lymphoma may be helpful to arrive at an accurate diagnosis and avoid unnecessary morbidity and mortality from inadvertent surgery.Main Messages• 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.

A case of primary pancreatic Burkitt's lymphoma diagnosed by EUS-guided FNA

A case of primary pancreatic Burkitt's lymphoma diagnosed by EUS-guided FNA

Primary Pancreatic Lymphoma

Primary pancreatic lymphoma (PPL) is a rare disease, accounting for only 0.5% of all pancreatic masses. A paucity of literature exists on the epidemiology and outcomes of PPL. Here, we present a series of 523 cases of PPL obtained from the Surveillance, Epidemiology, and End Results database. Patients diagnosed with a PPL from 1973 to 2007 were identified. Data on patient and tumor characteristics as well as initial treatment with surgery or radiation were extracted. Survival rates were calculated using the Kaplan-Meier method. A multivariate analysis was performed to determine independent prognostic factors predicting mortality hazard ratios using Cox proportional hazards modeling. Fifty-eight percent of patients identified were male. The median age range at diagnosis was 65 to 69 years. The most common histologic subtype in the present series was diffuse large B-cell lymphoma, which accounted for 56% of all patients. The 5-year overall survival for the group was 45%. Multivariate analysis suggests that age more than 60 years at diagnosis, race of "other" (compared with "white"), and marital status of single or widowed were predictive of increased all-cause mortality (P<0.05). This represents the largest published series of patients with PPL. Age more than 60 years, female sex, and marital status of married were identified as independent prognostic factors predicting for decreased all-cause mortality.

An Unusual Case of Synchronous Primary Pancreatic Lymphoma and Chronic Myeloid Leukemia

Purpose: Background: Non-Hodgkin's lymphoma occurring concurrently with chronic myelogenous leukemia (CML) is rare. Primary pancreatic lymphoma (PPL) is an even rarer type of extranodal non-Hodgkin's lymphoma originating from the pancreas. We report a unique case of de-novo pancreatic lymphoma developing synchronously in a patient on treatment for chronic myeloid leukemia. Case: A 61-year-old male presented with 2 weeks of abdominal pain, fatigue and weight loss of 10 lbs. Three days earlier, he developed darkening of his urine and vomiting. Three years prior, he was diagnosed with obstructive jaundice and found to have a pancreatic head mass which consisted mostly of inflammatory cells on fine needle aspiration (FNA) biopsy. He was managed conservatively with antibiotics and the mass resolved. Two years later, he was diagnosed with chronic myelogenous leukemia in accelerated phase and had achieved remission on Imatinib therapy. His physical examination was unremarkable. Labs were notable for elevated total bilirubin (2.8 mg/dl), aspartate transaminase (163 IU/L), alanine transaminase (218 IU/L) and alkaline phosphatase (468 IU/L). A computed tomography (CT) of the abdomen showed a 7.7 cm hypodense mass in the head of the pancreas with vascular encasement, peripancreatic mesenteric lymphadenopathy and extrahepatic ductal dilatation. CA 19-9 and CEA were within normal limits. An endoscopic ultrasonography confirmed a pancreatic head mass and FNA was consistent with a malignant lymphoma of follicle center origin with a clonal population of B lymphocytes. Discussion: Extranodal non-Hodgkin's lymphomas (NHL) represent up to 30-40% of all NHL cases with the gastrointestinal tract being the most commonly involved site. Although secondary involvement of the pancreas is not uncommon and well documented, PPL is an extremely rare disease, representing fewer than 2% of extranodal malignant lymphomas and 0.5% of all pancreatic masses. To our knowledge, this would represent the second reported case of synchronous primary extra nodal non-Hodgkin's lymphoma and CML. We present this unique case of synchronous non-Hodgkin's lymphoma and CML to highlight this unusual combination and to emphasize the diagnostic challenges posed by evaluation of pancreatic masses. It is equally important to maintain a low index of suspicion for lymphomatous involvement of the pancreas in middle-aged adults as the treatment and prognosis differs from adenocarcinoma.

Primary Pancreatic Lymphoma

Díaz-Nieto, Rafael MD, PhD; Olmos-Juárez, Erika MD; Pérez-Villa, Lidia MD; Estrada-Canjura, Dennis MD; Torres-Sierra, Juan MD; de Luna-Díaz, Resi MD Author Information

Primary Pancreatic Lymphoma in a Patient with Maturity Onset Diabetes of the Young type 3

Primary pancreatic lymphoma (PPL) is an extremely rare disease which occurs in pancreas, accounts for less than 1% of extra-nodal malignant lymphomas and 0,5% of cases of pancreatic masses. We report the case of PPL in a 15 year-old boy suffering from Maturity Onset Diabetes of the Young type 3 (MODY3) diagnosed at the age of 1 year.

A Rare Case of Primary Pancreatic Lymphoma

A Rare Case of Primary Pancreatic Lymphoma

Laparoscopic Multivisceral Resection for Infiltrating Primary Pancreatic Non-Hodgkin's Lymphoma Mimicking a Vast Cystic Neoplasm

Laparoscopic Multivisceral Resection for Infiltrating Primary Pancreatic Non-Hodgkin's Lymphoma Mimicking a Vast Cystic Neoplasm

Guide to diagnosing primary pancreatic lymphoma, B‐cell type: Immunocytochemistry improves the diagnostic accuracy of endoscopic ultrasonography‐guided fine needle aspiration cytology

Primary pancreatic lymphoma (PPL) is a rare disease with <1%of extranodal non-Hodgkin's lymphoma arising in the pancreas. This report provides immunocytochemical information on PPL that would be valuable for making differential diagnoses between PPL, pancreatic neuroendocine tumor, acinar cell carcinoma, and pancreatic ductal cancer. A 68-year-old woman had a chief complaint of abdominal pain. Fine needle aspiration cytology (FNAC)was performed. The FNAC smear showed moderate cellularity,with a small to moderate number of irregular cells and lymphocytes.No epithelial tumor clusters or abundant mucoid background were seen. The cells were scattered with pleomorphism and showed irregular nuclear shapes with finely granular chromatin,an increased nuclei–cytoplasm ratio, and prominent nucleoli.Cytologically, PPL was suspected with Papanicolaou staining but definite diagnosis was not made. Therefore, the specimen was destained, immunocytochemically examined for leukocyte common antigen (LCA), and PPL was suspected again. Numerous tumor cells were found in the surgical sample and tumor cells were positive for CD20 and negative for CD45RO. Based on these findings,the tumor was diagnosed as PPL, B-cell type. The preoperative FNAC smear that was examined for LCA was then reexamined for CD20, CEA, and Synaptophysin. As a result, the tumor cells were positive for LCA and CD20, whereas they were negative for CEA and Synaptophysin. Taking these findings together with the cytopathologic findings, this specimen was reconfirmed as PPL. Immunocytochemical examination for LCA and CD20 is useful in the identification of malignant pancreatic lymphoma, B-cell type.

Primary pancreatic lymphoma: A population-based analysis using the Surveillance, Epidemiology and End Results database.

303 Background: Primary pancreatic lymphoma (PPL) is a rare disease, accounting for only 0.5% of all pancreatic masses. The clinical presentation of PPL is similar to that of pancreatic adenocarcinoma and the two are difficult to distinguish radiographically. A paucity of literature exists on the epidemiology and outcomes of PPL. Given the limited case reports published on PPL, a comparison of the different treatment modalities has previously not been possible. Here, we present a series of 523 cases of PPL obtained from the Surveillance, Epidemiology, and End Results (SEER) database to investigate the tumor characteristics and compare the different treatment modalities. Methods: Patients diagnosed with a primary pancreatic lymphoma from 1973-2007 were identified. Data on patient and tumor characteristics as well as initial treatment with surgery or radiation was extracted. Chemotherapy information is not available through SEER. Overall survival was calculated using the Kaplan-Meier method. A multivariate analysis was performed to determine independent prognostic factors predicting for survival using a Cox proportional hazards model. Results: Fifty-eight percent of patients identified were male. The median age range at diagnosis was 65-69 years (range, 10-14 years – greater than 85 years). The most common histologic subtype in the present series was diffuse large B-cell lymphoma (DLBCL), which accounted for 71% of all patients. The 5-year overall survival for the group was 47%. Multivariate analysis indicates that age &gt;60 and a marital status of single were predictive of a decreased cause-specific and overall-survival specific survival (p&lt;0.05). Radiation therapy, but not surgery, was predictive of an improved overall survival (p&lt;0.05). Conclusions: PPL is a rare form of extra-nodal NHLs of the GI tract. Adjuvant radiation therapy for patients with a PPL should be strongly considered and a surgical treatment should be avoided if an early diagnosis is established. A prospective study evaluating this patient population will be difficult given the rarity PPL. We hope that this case series will provide a context in evaluating and treating patients with PPL. No significant financial relationships to disclose.

Primary Pancreatic Lymphoma

To investigate the clinical feature and treatment strategy of primary pancreatic lymphoma. Thirty-nine cases of primary pancreatic lymphoma reported in China were reviewed retrospectively with their clinical characters, treatment, and outcome, as well as a literature review of worldwide reports. The major clinical presentations included discomfort or pain in the upper abdomen and jaundice without specificity. Only 2 cases were identified correctly by computed tomography, and 5 cases obtained a positive finding in a biopsy before operation. Thirty-two patients accepted operation; 13 pancreatoduodenectomy and 6 distal pancreatectomy were performed. Thirty-one patients accepted postoperative chemotherapy. Until now, 26 patients are still alive at a range of 3 to 72 months; 5 patients died at 5 to 24 months after operation. Literature review revealed 85 additional cases of pancreatic lymphoma in English reports. Their diagnosis and treatment methods varied. Primary pancreatic lymphoma was misdiagnosed as pancreatic adenocarcinoma frequently. Fine needle aspiration biopsy is the most valuable method in preoperative diagnosis. The cyclophosphamide, doxorubicin, vincristine, and prednisone scheme was still the most commonly used regimen of chemotherapy. The value of surgery and radiotherapy remains controversial; an operation combining chemotherapy seems to be an appropriate method of treatment for a patient in whom malignancy cannot be ruled out.

Primary Pancreatic Lymphoma: A Rare Cause of Massive Upper Gastrointestinal Hemorrhage

1Department of Pathology and Laboratory Medicine; 2Department of Surgery; 3Department of Community Health Sciences; 4Department of Medicine, University of Calgary, Calgary, Alberta Correspondence: Dr Marc P Dupre, Department of Pathology and Laboratory Medicine, 3500 – 26th Avenue Northeast, Calgary, Alberta T1Y 6J4. Telephone 403-943-4036, fax 403-291-2931, e-mail mpdupre@gmail.com Received for publication May 27, 2011. Accepted May 29, 2011 Case Presentation A 68-year-old man presented with abdominal pain radiating to the back associated with a 25 kg weight loss. Abdominal ultrasound and computed tomography (CT) revealed an 8 cm partially cystic mass in the pancreatic head that encased the aorta and inferior vena cava (IVC) (Figure 1). A CT-guided fine-needle aspiration (FNA) was performed and confirmed a diagnosis of diffuse large B cell lymphoma. Subsequent staging CT demonstrated peripancreatic lymphadenopathy and air within the tumour, suggesting a possible communication between the intestine and the pancreatic mass. Direct visualization with gastroscopy confirmed a large penetrating ulcer between the second portion of the duodenum and the pancreatic tumour (Figure 2). Chemotherapy was delayed while pyloric exclusion surgery was performed to reduce the risk of chemotherapy-related uncontained perforation of the duodenum. The postoperative clinical course was complicated by a slowly declining hemoglobin level, followed by cardiopulmonary arrest in concert with a sudden drop in hemoglobin level to 50 g/L. An emergent upper endoscopy revealed copious amounts of blood within the stomach. The grave clinical deterioration precluded further resuscitative efforts, and the patient died from a massive upper gastrointestinal hemorrhage. A postmortem examination confirmed an 8 cm, extensively necrotic mass in the head of the pancreas. Significant peripancreatic lymphadenopathy was present; however, all nonregional lymph nodes were of normal size. The pancreatic head mass extended laterally and eroded into the adjacent duodenal wall producing the large mucosal erosion identified endoscopically. Posteriorly, the mass encased the aorta and the IVC, producing an irregular ulceration of the endolumenal surface of the IVC (Figure 3). This ulceration of the IVC resulted in a fistulous tract between the IVC and the duodenum via an intermediary of the pancreatic head mass. Histological examination revealed a poorly differentiated malignant neoplasm, which by immunohistochemical studies confirmed the original diagnosis of a diffuse large B cell lymphoma.

Primary Pancreatic Lymphoma

AbstractAbstract 4150 Introduction:Primary extranodal pancreatic non-Hodgkin lymphoma (PPL) makes up no more than 0.16–4.9% of pancreatic malignancies and less than 0.7% of non-Hodgkin's lymphomas (NHL). Since lymphomas involving the pancreas may have a similar clinical presentation to primary pancreatic adenocarcinoma, and often have a similar radiographic appearance, pancreatic lymphoma is often not diagnosed until surgical exploration or definitive surgery for presumed pancreatic cancer. Preoperative distinction of adenocarcinoma and PPL is critical as the management and prognoses of these malignancies are mutually exclusive. The rarity of PPL has made epidemiologic studies difficult to conduct. Methods:We queried our IRB-approved clinicopathologic database, derived from comprehensive tumor registry data at the Massachusetts General Hospital, for all adult patients diagnosed with PPL between 2000 and 2010. The database contains 5821 patients with mature lymphoid malignancies. Cases were included in the analysis if they met clinicopathologic criteria for PPL, defined as dominant disease presentation within the body of the pancreas. Forty-five patients were found to have pancreatic involvement at initial presentation of whom 31 (68.9%) on further investigation had a pancreatic primary and are included in the analysis. For each patient, we collected complete demographic information, clinical presenting features, histology, chemotherapy regimens, use of radiotherapy, and type of surgical biopsy performed. We also collected outcome data including results of interim and final restaging scans. Results:PPL represented 0.5% of all mature lymphomas seen at our institution. The median age at diagnosis was 60 yrs (range 20–91). There were 21 male and 10 female patients. Eighteen patients had Diffuse Large B Cell Lymphoma (DLBCL) (one with a focus of follicular lymphoma (FL) grade 3), two each had Burkitts Lymphoma, FL Grade 1–2, and mantle cell lymphoma (MCL), and one each had small lymphocytic lymphoma (SLL), Hodgkin Lymphoma, Marginal zone lymphoma, and peripheral T cell Lymphoma not otherwise specified. Three patients had NHL not otherwise specified (NOS). Of the 31 patients, 13 patients were stage 4E, 5 were stage 3E, 8 were stage 2E, and 5 were Stage 1E. Seventeen patients presented with jaundice. In 2 cases clinical history at presentation was unavailable. Elevated lactate dehydrogenase (LDH) level was present in 18 of 26 patients (69%) for whom laboratory values were available. B symptoms were present at diagnosis in 13 patients, absent in 17 patients, and unavailable in 1. Diagnosis was made in sixteen patients by fine needle aspiration (FNA), in nine patients by core needle biopsy, in two by incisional biopsies, and four patients were diagnosed after a definitive pancreaticoduodenectomy (Whipple procedure). Information on therapeutic regimen was available for 24 patients. One of these 24 patients was treated with primary radiotherapy without chemotherapy for a grade 1–2 primary pancreatic FL. Twenty-three patients had initial chemotherapy: 8 with R-CHOP, 5 with CHOP, 2 with CVP, and 1 each with CHOP-14, CVP × 1 then CHOP, R-CVP, R-EPOCH, R-CODOX-M/R-IVAC, R-CDOP and R-CP then R-CHOP. One patient was treated with chemotherapy not otherwise specified in the medical record. Four of these 23 patients received consolidative radiotherapy after initial therapy. Chemotherapy had significant efficacy with an overall response rate (ORR) of 75% in all chemotherapy treated patients (10 CR, 8 PR). Therapy information was available on 13 patients with DLBCL with an ORR of 85% (7 CR, 4 PR) to R-CHOP in 8 patients, CHOP in 3, and 1 each R-EPOCH and R-CDOP. One patient died of meningeal relapse of DLBCL despite therapy with R-EPOCH. For the entire PPL group at median follow-up of 28.5 months, the progression free and overall survivals are 48.4% and 64.5%, respectively. At a median follow-up of 33.4 months, the progression free and overall survivals for the DLBCL-PPL group are 50.0% and 66.7%, respectively. Discussion:PPL is a rare extranodal presentation of lymphoma accounting for 0.5% of lymphomas seen at a tertiary referral center. Pre-surgical diagnosis of PPL is critical to avoidance of unnecessary major surgery. The outcome of PPL approximates the outcome of DLBCL in other sites. An increased rate of CNS relapse was not seen with presentations in this extranodal site. Disclosures:Hochberg:Biogen Idec: Speakers Bureau; Genentech: Speakers Bureau; Enzon Pharmaceuticals: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Amgen: Speakers Bureau; WorldCare: Membership on an entity's Board of Directors or advisory committees; Proventys: Consultancy.

Endoscopic Ultrasound-Guided Fine-Needle Aspiration With Liquid-Based Cytologic Preparation in the Diagnosis of Primary Pancreatic Lymphoma

The diagnosis subtyping of lymphoma on specimens collected by endoscopic ultrasound fine-needle aspiration (EUS-FNA) can be extremely difficult. When a cytopathologist is available for the on-site evaluation, the diagnosis may be achieved by applying flow cytometric techniques. We describe our experience with immunocytochemistry (ICC) and molecular biology studies applied on EUS-FNA specimens processed with a liquid-based cytologic (LBC) preparation for the diagnosis of primary pancreatic lymphoma (PPL). Three patients with a pancreatic mass underwent EUS-FNA. The collected specimens were processed with the ThinPrep method for the cytologic diagnosis and eventual additional investigations. A morphologic picture consistent with PPL was found on the LBC specimens of the 3 patients. Subsequent ICC and molecular biology studies for immunoglobulin heavy chain gene rearrangement established the diagnosis of pancreatic large B-cell non-Hodgkin lymphoma in 2 patients and a non-Hodgkin lymphoma with plasmoblastic/immunoblastic differentiation in the remaining one. An LBC preparation can be used to diagnose and subtype PPL by applying ICC and molecular biology techniques to specimens collected with EUS-FNA. This method can be an additional processing method for EUS-FNA specimens in centers where on-site cytopathologist expertise is not available.

Linfoma pancreático primario diagnosticado mediante punción aspiración con aguja guiada por ultrasonografía endoscópica

Although primary pancreatic lymphoma (PPL) is a rare malignant tumor, the correct diagnosis is essential since their therapeutic management differs from other pancreatic tumors. The fine needle aspiration cytology guided by endoscopic ultrasonography (EUS-FNA) is currently the preferred technique for the diagnosis of neoplasms of the pancreas, being of particular interest in those pancreatic lesions with atypical characteristics or presentation. However, the usefulness of EUS-FNA in the PPL has been poorly studied because of the rarity of this entity. We report a case of a patient with HIV infection and PPL diagnosed by EUS-FNA.

Primary Pancreatic Lymphoma Mimicking Adenocarcinoma of Pancrease

Primary Pancreatic Lymphoma Mimicking Adenocarcinoma of Pancrease

Unique intense uptake demonstrated by 18F-FDG positron emission tomography/computed tomography in primary pancreatic lymphoma: A case report

Patients with primary pancreatic lymphoma (PPL), which is rare, require a different therapeutic approach and have a better prognosis than those with pancreatic cancer. However, conventional imaging modalities alone are not able to differentiate between pancreatic cancer and other rare tumors such as PPL, although the accurate diagnosis of PPL is crucial. The development of new modalities such as F-18 2'-deoxy-2fluoro-D-glucose (FDG) positron emission tomography combined with computed tomography (PET/CT) contributes to the evaluation of lymphoma staging. However, few reports are currently available regarding PET/CT findings in PPL. In this study, a 56-year old man with PPL was examined using FDG PET/CT imaging, which showed the unique intense uptake of FDG in the pancreas with atypical findings of malignancy in the CT scan and magnetic resonance images.

Primary Pancreatic Follicular Lymphoma Mimicking Adenocarcinoma

A 73-year-old woman presented with painless jaundice without fever, chills, or night sweats. Clinical examination revealed jaundice without stigmata of chronic liver disease, peripheral lymphadenopathy, abdominal mass, or hepatosplenomegaly. Laboratory studies were remarkable for a total bilirubin level of 19.1 mg/dL (normal, 0.1–1.0 mg/dL), direct bilirubin level of 14 mg/dL (normal, 0.0–0.3 mg/dL), and alkaline phosphatase level of 753 U/L (normal, 41–108 U/L); serum transaminase levels were mildly increased.

Endoscopic ultrasound-guided fine-needle aspiration with or without flow cytometry for the diagnosis of primary pancreatic lymphoma – a case series

The role of endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) with flow cytometry for the diagnosis of primary pancreatic lymphoma (PPL) has not been previously described. Our aims were to describe the EUS features of PPL and the role of EUS-FNA with and without flow cytometry in the diagnosis of 16 patients. When EUS-FNA with flow cytometry was compared with EUS-FNA without flow cytometry, the sensitivities for diagnosing non-Hodgkin's lymphoma were 84.6 % versus 30.8 %, respectively ( P = 0.01). EUS-FNA with flow cytometry is a valuable tool to diagnose PPL. Flow cytometry analysis complements traditional assessment by standard cytology.

Primary Pancreatic Lymphoma with Severe Dilatation of Pancreatic Duct: A Case Report

Primary pancreatic lymphoma is extremely rare and usually consists of a bulky tumor without internal necrosis and severe dilatation of the pancreatic duct. We report the unusual CT and MRI findings of a primary pancreatic lymphoma in a 27-years-old man, associated with severe dilatation of the pancreatic duct, common bile duct, and internal necrosis.