Acute pancreatitis as an unusual presentation of primary splenic lymphoma

Abstract

A 51-year-old man with no relevant medical history presents to the emergency department complaining of a recent worsening of few months upper abdominal pain with back radiation and postprandial fullness, without B symptoms. Laboratory analysis showed hyperamylasaemia, elevated lactate dehydrogenase and inflammatory parameters. Abdominal ultrasonography revealed a heterogeneous solid mass in the spleen/splenic hilum with pancreatic parenchyma continuity and no biliary tract dilation or gallstones. A mild acalculous acute pancreatitis diagnosis was made. Abdominopelvic CT revealed a large heterogeneous mass infiltrating the spleen and pancreas and obstructing the common bile duct at the pancreatic level with upstream dilation of biliary and pancreatic ducts, with splenic vein invasion. Several necrotic, peripancreatic and hepatic hilar adenopathies were also observed. Ultrasound-guided biopsy showed a primary splenic diffuse large B-cell non-Hodgkin's lymphoma. Chemotherapy with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone) was performed with lack of response and the patient died within 10 months of diagnosis onset.

Keywords: chemotherapy; haematology (including blood transfusion); malignant and benign haematology; pancreas and biliary tract; pancreatitis.

Figure 1

Abdominal ultrasonography showed a heterogeneous solid mass of approximately 11 cm×10 cm in dimension, without internal vascularisation, located in the upper pole of the spleen/splenic hilum with apparent pancreatic parenchyma continuity, and no biliary tract dilation, gallstones or peritoneal effusion.

Figure 2

Abdominopelvic CT (A: axial view, B: coronal view) showing a large heterogeneous lobulated mass, with 9.5 cm×13 cm×13 cm in dimension, infiltrating the spleen and pancreas, splenic vein invasion, multiple necrotic, peripancreatic and hepatic hilar adenopathies (10–32 mm) conditioning dilation of the Wirsung and intrahepatic and extrahepatic bile ducts with abrupt ending at the pancreatic level of the common bile duct, collateral venous circulation and mild peritoneal effusion.

Figure 3

Ultrasound-guided percutaneous biopsy of the splenic mass revealed a monotonous population of intermediate-to-large in size cells, with rare necrosis or apoptosis (A, H&E 100×). Immunohistochemical study was positive for CD20 (B, H&E 40×; C, H&E 100×), Bcl6, MUM1 and Ki67 (>80%), and negative for BCL2, CD10 and CD3, compatible with a primary splenic diffuse large B-cell non-Hodgkin’s lymphoma.

Figure 4

18F-FDG (fluorodeoxyglucose) positron emission tomography surveillance at 3 months (A) and 6 months (B) showed a hypermetabolic splenic lesion with dimension reduction (Deauville score 5).

Figure 5

Left arm soft tissue ultrasonography showed a 7.2 cm heterogeneous mass with multiple hypoechoic nodules and marked internal vascularity.