ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI A B-wildering Case of Recurrent Acute Pancreatitis Chandraprakash Umapathy,1 Mahesh Gajendran,1 and Jennifer S. Chennat2 1 Division of General Internal Medicine, and 2Division of Gastroenterology, Hepatology, and Nutrition, University of Pittsburgh, Pittsburgh, Pennsylvania

Question: A 46year-old man with a 3-month history of recurrent pancreatitis of unknown etiology was admitted for another episode of acute pancreatitis. Liver function tests (LFTs) at the time of initial presentation showed elevated bilirubin (4.5 mg/dL) and transaminase levels >200 U/L. CT scan and MR cholangiopancreatography (MRCP) revealed pancreatic inflammation, gallbladder wall thickening, and 0.9 cm common bile duct (CBD) dilatation. He underwent endoscopic retrograde cholangiopancreatography (ERCP) with biliary sphincterotomy and sludge removal (Figure A). Cholecystectomy was performed, but he developed postprandial abdominal pain and increased lipase levels in the postoperative period. Repeat MRCP showed worsening biliary and pancreatic duct obstruction, so he underwent repeat ERCP and a stent placement for mid-CBD stricture with brushing negative for malignant cells. He continued to have abdominal pain and an endoscopic ultrasonography (EUS) showed diffusely hypoechoic pancreatic parenchyma with fullness in pancreatic head (Figure B) along with hypoechoic lymph nodes with well-defined margins. Fine needle aspiration of the pancreas (25-G ProCore needle) showed negative immunoglobulin (Ig)G4 staining and no malignant cells on cytology. An ERCP was attempted, but failed owing to extrinsic compression of the duodenum that prevented passage of the endoscope. A percutaneous transhepatic cholangiogram (PTC) showed moderately severe intrahepatic biliary dilatation with abrupt occlusion of the CBD and an internal/external biliary catheter was placed, followed by improvement in LFTs. Despite this treatment, he continued to do poorly with persistent abdominal pain so he underwent repeat CT abdomen with contrast, which showed extensive infiltrating soft tissue attenuation extending from the pancreatic head into the root of the small bowel mesentery and along retroperitoneum. Soft tissue attenuation was inseparable from an enlarged, heterogeneous pancreatic head (Figure C). The descending duodenum, inferior vena cava, left renal vein, distal superior mesenteric vein, distal splenic vein and proximal main portal vein were markedly compressed by soft tissue (Figure D). A

Gastroenterology 2015;149:e9–e11

ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI second EUS was done and showed pancreatic duct stricture in the pancreatic head with dilation of 4.5mm in the mid-head region. Fine needle aspiration of the transition point was performed but cytology was nondiagnostic. What is the diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.

Conflicts of interest The authors disclose no conflicts. © 2015 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2014.12.038

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ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Answer to Image 5: B-Cell Lymphoma

Eventually, the patient underwent diagnostic laparoscopy with peritoneal lavage, partial portal lymphadenectomy, pancreatic biopsy, and mesenteric lymph node biopsy. Immunohistochemical stains and flow cytometric immunophenotypic studies of the lymph node and root of mesentery confirmed a CD20-positive, diffuse, large B-cell lymphoma with a germinal center phenotype (Figure E, F). Cytogenetic fluorescence in situ hybridization studies confirmed that the diffuse, large, B-cell lymphoma represented a “double-hit” transformation of the follicular lymphoma. The patient was discharged with a PTC catheter in situ for prolonged biliary drainage. He was started on chemotherapy with R-CHOP and then transitioned to the Hyper-CVAD regimen, which he is still receiving. Lymphoma is a rare cause of pancreatitis.1 Diffuse, large B-cell lymphoma accounts for 30% of newly diagnosed cases of non-Hodgkin lymphoma (NHL)2. Enlarged lymph nodes can cause extrahepatic bile duct obstruction resulting in jaundice and/or pancreatitis.2,3 NHL accounts for 1%-2% of cases of malignant biliary obstruction.3 In cases where extrahepatic bile duct obstruction is the initial manifestation of lymphoma, diagnosis can be challenging because these lesions can resemble benign stricture, infectious cholangitis, sclerosing cholangitis, or cholangiocarcinoma during cholangiography.3 CT is the most common modality used in diagnosing pancreatic and peripancreatic masses.2 Lymphoma can take one of two forms on imaging: a localized, well-circumscribed or diffuse, infiltrative hypoattenuating mass involving the pancreas.2 EUS-guided biopsy is safe and accurate, and can help with histologic confirmation of diagnosis, thereby avoiding surgery.1 In our patient, EUS-guided biopsy failed to diagnose the condition because patient did not have pancreatic involvement of the lymphoma. Patients presenting with biliary obstruction due to lymphoma are often in advanced stage of the disease.3 Chemotherapy is the mainstay of treatment,3 but often stent placement is required to relieve obstruction in the setting of biliary obstruction. Biliary drainage by endoscopic or percutaneous route is preferable to surgical biliary diversion, as a bridge to curative treatment of the lymphoma.3 Lymphoma, although uncommon, should be considered as a cause of malignant biliary obstruction, particularly in younger patients, because it can be very sensitive to chemotherapy and radiation.

References 1. 2. 3.

Anderson JH, Morran CG, Anderson JR, et al. Acute pancreatitis and non-Hodgkin’s lymphoma. Postgrad Med J 1987; 63:137–139. Saif MW, Khubchandani S, Walczak M. Secondary pancreatic involvement by a diffuse large B-cell lymphoma presenting as acute pancreatitis. World J Gastroenterol 2007;13:4909–4911. Odemis B, Parlak E, Basar O, et al. Biliary tract obstruction secondary to malignant lymphoma: experience at a referral center. Dig Dis Sci 2007;52:2323–2332.

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A B-wildering Case of Recurrent Acute Pancreatitis.

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