Pancreatic Lymphoma Research Articles

Pictorial Review of Rare Pancreatic Tumors and Tumor-like Lesions: Radiologic–Pathologic Correlation

Rare pancreatic tumors and non-neoplastic tumor-like lesions present a diagnostic challenge due to their uncommon occurrence and overlapping imaging characteristics with more prevalent pancreatic neoplasms. Advances in imaging technologies and diagnostic criteria have contributed to increased detection of these rare entities in clinical practice. This pictorial review focuses on the radiologic–pathologic correlation of rare pancreatic tumors, including colloid carcinoma, acinar cell carcinoma, pancreatoblastoma, primary pancreatic lymphoma, and non-neoplastic tumor-like lesions such as hamartomas and inflammatory pseudotumors. Detailed imaging features, such as signal intensities on MRI and enhancement patterns on CT, are correlated with pathological findings to assist in the differential diagnosis. Familiarity with these characteristics is crucial for radiologists to ensure accurate diagnosis and guide appropriate treatment strategies, as management and prognosis significantly differ from common pancreatic neoplasms.

Plea to radiologists: Please consider Mahvash disease when encountering an enlarged pancreas.

Radiologists play a key role in establishing an early and accurate diagnosis, especially for rare diseases. Mahvash disease (OMIM 619290) is an autosomal recessive hereditary disease caused by inactivating mutations of the glucagon receptor and its main clinical consequences are pancreatic neuroendocrine tumors and in some cases, porto-sinusoidal vascular disease and portal hypertension. Untreated Mahvash disease can be lethal. The diagnosis of Mahvash disease has almost always been delayed in the past due to radiologists' unawareness of or unfamiliarity with the unique imaging features of Mahvash disease which are moderately to enormously enlarge pancreas with preserved pancreas contour and parenchyma without vascular involvement or lymphadenopathy. These features help differentiate Mahvash disease from other etiologies of diffusely enlarged pancreas such as diffuse pancreatic ductal carcinoma, diffuse pancreatic lymphoma, and autoimmune pancreatitis. Invoking Mahvash disease in the differential diagnosis of an enlarged pancreas has recently been shown to facilitate early diagnosis. To prevent missing the diagnosis of this significant disease, I sincerely ask radiologists to consider Mahvash disease in their differential diagnoses of diffusely enlarged pancreas.

Primary Pancreatic Lymphoma with Active Gastric Bleeding. A Clinical Case and Review Literature.

Primary Pancreatic Lymphoma with Active Gastric Bleeding. A Clinical Case and Review Literature.

Preliminary study on the ability of the machine learning models based on 18F-FDG PET/CT to differentiate between mass-forming pancreatic lymphoma and pancreatic carcinoma

PurposeThe objective of this study was to preliminarily assess the ability of metabolic parameters and radiomics derived from 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) to distinguish mass-forming pancreatic lymphoma from pancreatic carcinoma using machine learning. MethodsA total of 88 lesions from 86 patients diagnosed as mass-forming pancreatic lymphoma or pancreatic carcinoma were included and randomly divided into a training set and a validation set at a 4-to-1 ratio. The segmentation of regions of interest was performed using ITK-SNAP software, PET metabolic parameters and radiomics features were extracted using 3Dslicer and PYTHON. Following the selection of optimal metabolic parameters and radiomics features, Logistic regression (LR), support vector machine (SVM), and random forest (RF) models were constructed for PET metabolic parameters, CT radiomics, PET radiomics, and PET/CT radiomics. Model performance was assessed in terms of area under the curve (AUC), accuracy, sensitivity, and specificity in both the training and validation sets. ResultsStrong discriminative ability observed in all models, with AUC values ranging from 0.727 to 0.978. The highest performance exhibited by the combined PET and CT radiomics features. AUC values for PET/CT radiomics models in the training set were LR 0.994, SVM 0.994, RF 0.989. In the validation set, AUC values were LR 0.909, SVM 0.883, RF 0.844. ConclusionMachine learning models utilizing the metabolic parameters and radiomics of 18F-FDG PET/CT show promise in distinguishing between pancreatic carcinoma and mass-forming pancreatic lymphoma. Further validation on a larger cohort is necessary before practical implementation in clinical settings.

Primary pancreatic lymphoma: Histopathological pattern of 8 cases.

To report the histopathological pattern of primary pancreatic lymphoma (PPL) in 2 tertiary hospitals. The pathology slides and reports of all the cases diagnosed in pathology departments in 2 referral hospitals were reviewed. An additional immunohistochemistry study was done to reclassify lymphomas according to the current system. Eight patients with PPL have been identified. The ages ranged from 36 to 71 years. Clinical presentation includes abdominal pain, weight loss, jaundice, abdominal mass, nausea, and vomiting. Pathological evaluation revealed 5 diffuse large B-cell lymphomas, one high-grade B-cell lymphoma, one MALT lymphoma, and one follicular lymphoma. Primary pancreatic lymphoma is a very rare tumor without specific clinical, laboratory tests, or radiological findings. Abdominal pain is the most common clinical presentation. Diffuse large b-cell lymphoma is the most common pathological subtype. Primary pancreatic lymphoma should be taken into consideration when evaluating pancreatic mass to avoid unnecessary surgical resection.

Complementary comments on diagnosis, severity and prognosis prediction of acute pancreatitis.

The radiological differential diagnosis of acute pancreatitis includes diffuse pancreatic lymphoma, diffuse autoimmune pancreatitis and groove located mass lesions that may mimic groove pancreatitis. Dual energy computed tomography and diffusion weighted magnetic resonance imaging are useful in the early diagnosis of acute pancreatitis, and dual energy computed tomography is also useful in severity assessment and prognosis prediction. Walled off necrosis is an important complication in terms of prognosis, and it is important to know its radiological findings and distinguish it from pseudocyst.

EPI-X4, a CXCR4 antagonist inhibits tumor growth in pancreatic cancer and lymphoma models

Endogenous peptide inhibitor for CXCR4 (EPI-X4) is a CXCR4 antagonist with potential for cancer therapy. It is a processed fragment of serum albumin from the hemofiltrate of dialysis patients. This study reports the efficacy of fifteen EPI-X4 derivatives in pancreatic cancer and lymphoma models. In vitro, the peptides were investigated for antiproliferation (cytotoxicity) by MTT assay. The mRNA expression for CXCR4 and CXCL12 was determined by RT-PCR, chip array and RNA sequencing. Chip array analysis yielded 634 genes associated with CXCR4/CXCL12 signaling. About 21% of these genes correlated with metastasis in the context of cell motility, proliferation, and survival. Expression levels of these genes were altered in pancreatic cancer (36%), lymphoma models (53%) and in patients’ data (58%). EPI-X4 derivatives failed to inhibit cell proliferation due to low expression of CXCR4 in vitro, but inhibited tumor growth in the bioassays with significant efficacy. In the pancreatic cancer model, EPI-X4a, f and k inhibited mean tumor growth by > 50% and even caused complete remissions. In the lymphoma model, EPI-X4b, n and p inhibited mean tumor growth by > 70% and caused stable disease. Given the non-toxic and non-immunogenic properties of EPI-X4, these findings underscore its status as a promising therapy of pancreatic cancer and lymphoma and warrant further studies. Simple summaryThis study examined the value of chemokine receptor CXCR4 as an antineoplastic target for the endogenous peptide inhibitor of CXCR4 (EPI-X4), a 12-meric peptide derived from serum albumin. EPI-X4 inhibits CXCR4 interaction with its natural ligand, CXCL12 (SDF1). Therefore, malignancies (including pancreatic cancer and lymphoma) that depend on the CXCR4/CXCL12 pathway for progression can be targeted with EPI-X4. Of 634 genes that were linked to the CXCR4/CXCL12 pathway, 21% were associated with metastasis. In cultured human Suit2–007 pancreatic cancer cells, CXCR4 showed low to undetectable expression, which was why EPI-X4 did not inhibit pancreatic cancer cell proliferation. These findings were different in vivo, where CXCR4 was highly expressed and EPI-X4 inhibited tumor growth in rodents harboring pancreatic cancer or lymphoma. In the pancreatic cancer model, EPI-X4 derivatives a, f and k caused complete remissions, while in lymphomas EPI-X4 derivatives b, n and p caused stable disease.

Primary Pancreatic Lymphoma: An Analysis of the National Cancer Database

Background: Primary pancreatic lymphoma (PPL) is a rare entity, accounting for fewer than 2% of extranodal malignant lymphomas and 0.5% of all pancreatic masses. Fewer than 150 cases of PPL have been reported in the English language medical literature. We aimed to utilize the National Cancer Database (NCDB) to investigate the clinicopathological features and prognostic factors in PPL. Methods: The NCDB for Non-Hodgkin lymphoma was used to identify 2130 patients diagnosed with PPL (2004 and 2020). The database was used to examine clinicopathological features. Survival analysis was done by Kaplan-Meier. Cox proportional hazards was adopted for prognostic variable evaluation. Results: Most PPL patients were males (58.5%) and white (87.7%), mean age at diagnosis was 67.38 years. Histologically 59.6% were diffuse large B-cell. 14.9% Follicular, 2.6 % Burkitt, & 15.8% Lymphoma NOS. Regarding treatment 71.5% of patients received chemotherapy. Immunotherapy given in 21.9%. Radiation in 12.1%, and 7.3% received some form of surgical treatment. Median survival was 85.32 months (CI 79.79-90.84). Using Cox hazard regression, the following factors were statistically significant (p<0.001): Age >60 (HR: 2.36,CI 1.9-2.8), increasing Charlson-Deyo Score, DLBL histology (HR:0.57,CI 0.45-0.72), stage 3 (HR:1.9,CI 1.6-2.2), Chemotherapy (HR 0.4,CI 0.34-0.46),and immunotherapy (HR 0.61,CI0.62-0.89) Conclusion: This is the most comprehensive study to date to utilize the NCDB to study the characteristics and prognostic factors of patients with PPL. DLBL histology, chemotherapy and immunotherapy are associated with reduced risk of death.

Pancreatic diffuse large B-cell lymphoma: a case report and literature review

Pancreatic diffuse large B-cell lymphoma: a case report and literature review

Primary pancreatic lymphoma in a child presenting with rapidly progressive obstructive jaundice

Primary pancreatic lymphoma in a child presenting with rapidly progressive obstructive jaundice

Primary Pancreatic Lymphoma: A Rare Clinical Entity

Pancreatic adenocarcinoma is one of the most aggressive malignant diseases, rarely, pancreatic lymphomas are diagnosed. The final diagnosis of primary pancreatic lymphoma demands histopathology of the lesion; however, radiological features and tumor markers can help to give an idea about the disease. The most important difference between primary pancreatic lymphomas and pancreatic adenocarcinoma is the different treatment modalities the response to treatment and finally good prognosis in primary pancreatic lymphoma cases. Clinically it is difficult to differentiate the symptoms of primary pancreatic lymphomas from other carcinomas of the pancreas. Rarely patients may experience B- symptoms of lymphomas that are night sweats and fever in the case of primary pancreatic lymphomas.

Rare Solid Pancreatic Lesions on Cross-Sectional Imaging.

Several solid lesions can be found within the pancreas mainly arising from the exocrine and endocrine pancreatic tissue. Among all pancreatic malignancies, the most common subtype is pancreatic ductal adenocarcinoma (PDAC), to a point that pancreatic cancer and PDAC are used interchangeably. But, in addition to PDAC, and to the other most common and well-known solid lesions, either related to benign conditions, such as pancreatitis, or not so benign, such as pancreatic neuroendocrine neoplasms (pNENs), there are solid pancreatic lesions considered rare due to their low incidence. These lesions may originate from a cell line with a differentiation other than exocrine/endocrine, such as from the nerve sheath as for pancreatic schwannoma or from mesenchymal cells as for solitary fibrous tumour. These rare solid pancreatic lesions may show a behaviour that ranges in a benign to highly aggressive malignant spectrum. This review includes cases of an intrapancreatic accessory spleen, pancreatic tuberculosis, solid serous cystadenoma, solid pseudopapillary tumour, pancreatic schwannoma, purely intraductal neuroendocrine tumour, pancreatic fibrous solitary tumour, acinar cell carcinoma, undifferentiated carcinoma with osteoclastic-like giant cells, adenosquamous carcinoma, colloid carcinoma of the pancreas, primary leiomyosarcoma of the pancreas, primary and secondary pancreatic lymphoma and metastases within the pancreas. Therefore, it is important to determine the correct diagnosis to ensure optimal patient management. Because of their rarity, their existence is less well known and, when depicted, in most cases incidentally, the correct diagnosis remains challenging. However, there are some typical imaging features present on cross-sectional imaging modalities that, taken into account with the clinical and biological context, contribute substantially to achieve the correct diagnosis.

Semaglutide and cancer: A systematic review and meta-analysis

BackgroundFrench national health care insurance system database has suggested 1–3 years use of glucagon like peptide-1 receptor agonists (GLP1RA) (exenatide, liraglutide and dulaglutide) may be linked with increased occurrence of thyroid cancer. Similar data on semaglutide is not-available. Hence, we undertook this systematic review to look at the safety of semaglutide focussing on different cancers. MethodsDatabases were searched for randomized controlled trials (RCTs) and real-world studies involving patients receiving semaglutide in the intervention-arm. Primary outcome was to evaluate the occurrence of pancreatic and thyroid cancers. Secondary outcomes were to the evaluate occurrence of any other malignancies or severe adverse-events. ResultsData from 37 RCTs and 19 real-world studies having 16,839 patients in placebo-control group, 16,550 patients in active-control group and 13,330 patients in real-world studies were analysed. Compared to placebo, occurrence of pancreatic cancer [OR 0.25 (95%CI: 0.03–2.24); P = 0.21], thyroid cancer [OR 2.04 (95%CI: 0.33–12.61); P = 0.44; I2 = 0%] and all neoplasms (benign, malignant and otherwise unspecified) [OR 0.95 (95%CI:0.62–1.45); P = 0.82; I2 = 0%] was similar in the semaglutide group. Compared to active controls, occurrence of pancreatic cancer [OR 0.40 (95%CI:0.09–1.87); P = 0.26; I2 = 0%], thyroid cancer [OR 1.19 (95%CI:0.15–9.66); P = 0.87; I2 = 0%] and all neoplasms (benign, malignant and otherwise unspecified) [OR 0.91 (95% CI: 0.44–1.89); P = 0.79; I2 = 0%] were similar in the semaglutide group. Real-world data analysis revealed single case each of pancreatic cancer and B-cell lymphoma. ConclusionSemaglutide use in RCTs and real-world studies was not associated with an increased risk of any types of cancer, and this conclusion is supported by a high grade of evidence.

Primary Pancreatic Lymphoma Masquerading as Carcinoma on 18 F-FDG PET/CT.

A 76-year-old man with yellowish discoloration of sclera and skin for 2 months was referred to 18 F-FDG PET/CT for metabolic characterization of the mass in the pancreas. The images showed intense FDG uptake in the head of the pancreas, as well as a lymph nodal mass in the hepatic hilar region, which was consistent with pancreatic malignancy. Histopathologic findings showed characteristic findings of diffuse large B-cell lymphoma with no evidence of adenocarcinoma.

Low-dose acetylsalicylic acid for cancer prevention considering risk factors: a retrospective cohort study

PurposeAspirin (acetylsalicylic acid) has been reported to protect against certain cancers. However, patient-related risk factors may moderate protective effects, including excess weight, smoking, risky alcohol use, and diabetes. We explore the cancer-risk relationship between aspirin intake and those four factors. MethodsRetrospective cohort study of cancers, aspirin intake, and four risk factors in persons aged ≥50 years. Participants received medication during 2007–2016, and cancers were diagnosed in 2012–2016. Adjusted hazard ratios (aHR) for 95% confidence intervals (95%CI) were calculated for aspirin intake and risk factors using Cox proportional hazard modeling. ResultsOf 118,548 participants, 15,793 consumed aspirin, and 4003 had cancer. Results indicated a significant protective effect of aspirin against colorectal (aHR: 0.7; 95%CI: 0.6–0.8), pancreatic (aHR: 0.5; 95%CI: 0.2–0.9), prostate (aHR: 0.6; 95%CI: 0.5–0.7) cancers and lymphomas (aHR: 0.5; 95%CI: 0.2–0.9), and also, although not significantly, against esophageal (aHR: 0.5; 95%CI: 0.2–1.8), stomach (aHR: 0.7; 95%CI: 0.4–1.3), liver (aHR: 0.7; 95%CI: 0.3–1.5), breast (aHR: 0.8; 95%CI: 0.6–1.0), and lung and bronchial (aHR: 0.9; 95%CI: 0.7–1.2) cancers. Aspirin intake was not significantly protective against leukemia (aHR: 1.0; 95%CI: 0.7–1.4) or bladder cancer (aHR: 1.0; 95%CI: 0.8–1.3). ConclusionsOur results suggest that aspirin intake is associated with a reduced incidence of colorectal, pancreatic, and prostate cancers and lymphomas.

Pancreatic Diffuse Large B-cell Lymphoma in the US Population.

Pancreatic lymphomas (PLs) represent <2% of all lymphomas and <0.5% of all pancreatic neoplasms. An accurate histologic diagnosis of PL is needed to predict prognosis and adequately treat the patient. This study aims to investigate the demographic, clinical, and pathological factors affecting the prognosis and survival of pancreatic diffuse large B-cell lymphoma (DLBCL). Demographic and clinical data from 493 cases of DLBCL of the pancreas were identified between 2000 and 2018 using the Surveillance, Epidemiology, and End Results (SEER) database. The most common age group was between the ages of 70 and 79 years (27.0%).While 44%of cases involved distant sites (a proxy for secondary pancreatic DLBCL), regional and localized involvement was seen in 33%, with the most common cause of death being a primary pancreatic DLBCL. Most patients (71%) received only chemotherapy (systemic therapy). The overall five-year observed survival was 46% (95% CI, 43.5-48.3). The one-year and five-year survival with chemotherapy only was 68% (95% CI, 65.3-70.3) and 48% (95% CI, 44.7-50.5), respectively. The one-year and five-year survival with surgery and chemotherapy was 96% (95% CI, 91.3-99.9) and 80% (95% CI, 71.4-89.2), respectively. Surgery with chemotherapy (HR: 0.397 (95% CI, 0.197-0.803), p = 0.010) were both positive predictors in survival prognosis. Multivariable analysis identified age >55 years (HR: 2.475 (95% CI, 1.770-3.461), p < 0.001), distant stage (HR: 6.894 (95% CI, 4.121-11.535), p < 0.001), and undergoing no surgery (HR: 2.610 (95% CI, 1.307-5.215), p = 0.007) as negative predictors for survival. PLs are rare malignant pancreatic neoplasms with DLBCL being the most common histological subtype. An accurate and timely diagnosis of pancreatic DLBCL is necessary to implement effective treatments and reduce mortality. Systemic therapy (chemotherapy) with or without surgical therapy improved survival. Increased age and regional and distant spread negatively impacted survival.

Autopsy of a Patient with Primary Pancreatic Lymphoma with Findings Resembling Severe Acute Pancreatitis.

Primary pancreatic lymphoma is a rare pancreatic malignancy, reportedly accounting for only 0.2-0.7% of all primary pancreatic tumors. Primary pancreatic lymphoma is often difficult to distinguish from other diseases, such as acute pancreatitis. We herein report the autopsy of a patient with primary pancreatic lymphoma with imaging findings resembling those of severe acute pancreatitis, with a focus on the gross and histological features.

Clinical features and images of malignant lymphoma localized in the pancreatic head to differentiate from pancreatic ductal adenocarcinoma: a case series study

BackgroundPathological examination by endoscopic ultrasonography–guided fine-needle aspiration (EUS-FNA) has been reported to be useful in diagnosing pancreatic malignant lymphoma (ML), but some ML cases are difficult to be differentiated from pancreatic ductal adenocarcinoma (PDAC).MethodsThis retrospective study included 8 patients diagnosed with ML that had a pancreatic-head lesion at initial diagnosis and 46 patients with resected PDAC in the pancreatic head between April 2006 and October 2021 at our institute. ML and PDAC were compared in terms of patients’ clinical features and imaging examinations.ResultsThe median tumor size was larger in ML than in PDAC (45.8 [24–64] vs. 23.9 [8–44] mm), but the median diameter of the caudal main pancreatic duct (MPD) was larger in PDAC (2.5 [1.0–3.5] vs. 7.1 [2.5–11.8] mm), both showing significant differences between these malignancies (both, P < 0.001). In the analysis of covariance, MLs showed a smaller caudal MPD per tumor size than PDACs, with a statistical difference (P = 0.042). Sensitivity and specificity using sIL-2R ≥ 658 U/mL plus CA19-9 < 37 U/mL for the differentiation of ML from PDAC were 80.0% and 95.6%, respectively.ConclusionsDiagnosing pancreatic ML using cytohistological examination through EUS-FNA can be difficult in some cases. Thus, ML should be suspected if a patient with a pancreatic tumor has a small MPD diameter per tumor size, high serum sIL-2R level, normal CA19-9 level. If the abovementioned features are present and still cannot be confirmed as PDAC, re-examination should be considered.

Primary pancreatic lymphoma and metastatic lymphoma cases diagnosed with ultrasonography guided tru-cut needle biopsy; Two case reports

Secondary pancreatic involvement can be seen up to 30% of advance staged non-Hodgkin’s lymphoma cases, but primary pancreatic lymphomas constitute only 1% of extranodal lymphomas. Furthermore, 0.2% of the pancreatic space-occupying lesions are primary pancreatic lymphomas, which usually present as a mass in the head of the pancreas with nonspecific symptoms. Therefore, primary pancreatic lymphomas should be considered as differential diagnosis of pancreatic solid lesions. Herein, we report two cases with different clinical presentation and different course of disease resulting in the diagnosis of primary and metastatic pancreatic lymphoma.

A case report of a collision tumor composed of pancreatic ductal adenocarcinoma and peri-pancreatic mucosa-associated lymphoid tissue lymphoma

BackgroundCollision tumors are composed of two distinct tumor components. Collision tumors composed of pancreatic ductal adenocarcinoma and malignant lymphoma occurring in the pancreas have not been previously described in the scientific literature. In this case report, we describe a unique patient with a collision tumor composed of pancreatic ductal adenocarcinoma and peri-pancreatic mucosa-associated lymphoid tissue (MALT) lymphoma occurring in the pancreas.Case presentationAn 82-year-old woman presented to our hospital complaining of dizziness. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a large lymphoid lesion spreading from the peri-pancreatic tissue heading to the hepatic hilar plate, involving the hepatoduodenal ligament and the entire duodenum, also showing a hard tumor in the pancreas head. We performed echo-guided needle biopsies for each tumor and diagnosed a collision tumor composed of pancreatic ductal adenocarcinoma and low-grade B cell lymphoma. The patient underwent pancreaticoduodenectomy. The resected specimen showed an elastic hard tumor, 90 × 75 mm in size, located in the pancreatic head, and a whitish-yellow hard tumor involving the lower bile duct, 31 mm in size, located in the center of the pancreatic head. Pathological and immunohistochemical examination proved that pancreatic ductal adenocarcinoma and MALT lymphoma originating from the peri-pancreatic head collided in the pancreatic head.ConclusionsTo best of our knowledge, this is the first report of a surgically resected collision tumor of pancreatic ductal adenocarcinoma and MALT lymphoma originating from the peri-pancreatic head. A needle biopsy is useful when inconsistent findings are observed on diagnostic CT and MRI of tumor lesions since there is the possibility of a collision tumor.