A 75-year-old woman presented with a three-month history of abdominal pain radiating to the back, nausea, vomiting and weight loss. Her past history was unremarkable except for diabetes of 5 years duration which was regulated with diet alone. Her laboratory tests upon admission were as follows: white blood cell count 20,700 mm-3 (reference range: 4,000-11,000 mm-3), hemoglobin 8.5 g/dL (reference range: 11.7-15.5 g/dL), lactate dehydrogenase 501 U/L (reference range: 240-480 U/L), glucose 216 mg/dL (reference range: 70-110 mg/dL), total protein 5.95 g/dL (reference range: 6.4-8.3), albumin 2.6 g/dL (reference range: 3.4-4.8 g/dL), beta-2-microglobulin 10,355 ng/mL (reference range: 609-2,366 ng/mL). The tumor marker levels of AFP, CEA; CA 125, CA 15-3 and CA 19-9 were all within the normal range. Abdominal computed tomography (Figure 1) revealed a mass in the pancreatic tail which could not be distinguished from the pancreas parenchyma and nearby surrounding soft tissues, with encasement of the splenic artery and vein, accompanied by splenic infarct and lymphadenopathies located at the portal hilus, para-aortic region and left renal hilus. To differentiate the origin of the pancreatic mass as an endocrine or an exocrine tumor, serotonin, 3-methoxytiramine, metanephrine, normetanephrine, vanyl mandelic acid, 5 hydroxy indole acetic acid and dopamine tests were performed and were found to be within normal levels. Percutaneous ultrasonography-guided biopsy of the suspicious peripancreatic node confirmed the diagnosis. Immunohistochemical studies of the biopsied tissue demonstrated large atypical lymphoid cells which were positive for CD20 with a Ki-67 index of 30%. The final diagnosis was diffuse large B cell lymphoma. Bone marrow aspiration and biopsy did not show any involvement of disease.
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A Case of Pancreatic LymphomaMichael Piesman, MD; David G. Forcione, MD; David L. Carr-Locke MD, FRCP, FACG, FASGE Series Editor: David L. Carr-Locke, MD, FRCP, FACG, FASGE|Disclosures CASE REPORTA 55-year-old man with a medical history significant for coronary artery disease and obstructive sleep apnea presented to the hospital with a 3-month history of abdominal pain and recent onset of bright red blood per rectum. Approximately 3 months before admission, the patient reported the onset of waxing and waning abdominal pain of increasing severity, which migrated from the right lower to right upper quadrant. Occasionally the pain occurred in the epigastrium and radiated to the back. He reported poor appetite, early satiety, and a 20-pound weight loss over the previous 2 months. He had had nausea, dry heaves, night sweats, and fatigue.One day before admission, the patient noted 2 loose bowel movements that turned the toilet water brownish-red. He has not had any bowel movements since this time. He denied prior hematochezia or melena, but had had small amounts of bright red blood on the toilet paper thought to be related to hemorrhoids. On physical examination, the patient had normal blood pressure and pulse rate; his abdomen was obese, soft, and nondistended. He had mild tenderness in the epigastric region with no peritoneal signs. His stool was guaiac positive. Laboratory analysis revealed a hematocrit drop from 42 % to 27 % over the past 3 months. The white blood cell count was within normal limits and lactate dehydrogenase was elevated to 2 times the normal level. Abdominal computed tomography (CT) scan demonstrated a 7 x 5-cm well-defined mass encasing the celiac axis and involving the head of the pancreas. Multiple enlarged lymph nodes were present adjacent to the mass (Figure 1).(Enlarge Image)Figure 1.The CT scan demonstrates a lobulated 7 x 5-cm mass centered in the region of the pancreatic head and celiac axis.Upper endoscopy revealed an ulcerated friable mass in the second portion of the duodenum with no active bleeding (Figures 2, 3). Endoscopic ultrasound examination revealed an approximately 4-cm well-defined hypoechoic homogeneous mass in the head of the pancreas (Figures 4, 5); with adjacent 2-cm well-defined hypoechoic lymph nodes (Figure 6).(Enlarge Image)Figure 2.Endoscopic view of an ulcerated mass in the second portion of the duodenum.(Enlarge Image)Figure 3.Another view of the ulcerated mass in the second portion of the duodenum.(Enlarge Image)Figure 4.Endoscopic ultrasound image of a 4 x 5-cm hypoechoic homogeneous mass (solid arrow) in the head of the pancreas.(Enlarge Image)Figure 5.Endoscopic ultrasound image demonstrating normal pancreatic parenchyma (solid arrow) adjacent to the hypoechoic mass. The pancreatic duct is 2.7 mm in diameter.(Enlarge Image)Figure 6.Endoscopic ultrasound image of a 2-cm well-defined hypoechoic lymph node adjacent to the pancreas.Fine-needle aspiration of the mass and the lymph node (Figure 7) was performed. Pathology demonstrated the presence of malignant lymphoma dominated by large cells; flow cytometry confirmed the presence of large B-cell lymphoma. The patient was treated with 4 cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) with complete resolution of the pancreatic mass.(Enlarge Image)Figure 7.Endoscopic ultrasound image demonstrating fine-needle aspiration of a hypoechoic lymph node.
Discussion
Primary pancreatic lymphoma is a rare nonepithelial tumor of the pancreas that can mimic pancreatic adenocarcinoma clinically and radiographically. Differentiating between the 2 malignancies is important because pancreatic lymphoma has a high cure rate
Instead of being primary in the gland, pancreatic lymphoma can also represent direct extension from adjacent peripancreatic lymphadenopathy.
Diagnostic criteria of primary pancreatic lymphoma include a pancreatic mass that predominates with gross involvement of only the peripancreatic lymph nodes, no hepatic or splenic involvement, no palpable superficial lymphadenopathy, no enlargement of the mediastinal lymph nodes on chest radiography, and a normal leukocyte count [1].
1. Behrns KE, Sarr MG, Strickler JG. Pancreatic lymphoma: is it a surgical disease? Pancreas 1994; 9:662-667
primary manifestation is rare. Fewer than 2% of extranodal non-Hodgkin's lymphomas arise in the pancreas [2, 3]
This patient fulfilled these three criteria. We believe that this patient had stage 4A disease because of the diffuse involvement of the pancreas with infiltration to the peripancreatic area. No nearby infiltrating adenopathies or tumors of the other organs were noted.
The head of the pancreas is the most common location for primary pancreatic lymphoma, but the entire organ may be affected. The majority of cases of primary pancreatic lymphoma are of the B-cell type, but some cases of T-cell pancreatic lymphoma have been described in a Japanese series [4].
4. Mulkeen AL, Yoo PS, Cha C. Less common neoplasms of the pancreas. World J Gastroenterol 2006; 12:3180-5.
Appearances on CT can be helpful to differentiate the two conditions, but are not definitive[9, 10]
9. Lawler LP, Horton KM, Fishman EK: Peripancreatic masses that simulate pancreatic disease: spectrum of disease and role of CT. Radiographics 2003, 23 (5) :1117–1131
10. Teefey SA, Stephens DH, Sheedy PF 2nd: CT appearance of primary pancreatic lymphoma.Gastrointest Radiol 1986, 11 (1) : 41–43.
Ca19-9 is the most useful tumour marker in pancreatic carcinoma, but can be misleading as it may also be elevated in other malignancies, particularly of the upper gastrointestinal tract, including PPL as described in case one [11]. Without definitive pathologic diagnosis, potentially curable conditions such as PPL; as well as other malignancies with more favourable prognosis, including periampullary, distal common bile duct, duodenal and mucinous cyst adenocarcinomas, may be misdiagnosed[12].
11. Mann DV, Edwards R, Ho S, Lau WY, Glazer G:Elevated tumour marker CA19–9: clinical interpretation and influence of obstructive jaundice. Eur J Surg Oncol 2000, 26 (5) : 474–479.
12. Hart MJ, White TT, Brown PC, Freeny PC: Potentially curable masses in the pancreas. Am J Surg 1987, 154(1) : 134–136.
To our knowledge, MR imaging findings in patients with primary pancreatic lymphoma are not reported in the literature
Figure 1. Abdominal computed tomography shows a mass of the pancreas invading the nearby surrounding soft tissues