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Lipase hyper-secretion syndrome (LHS) is a rare para-neoplastic syndrome that has been reported in association with acinar cell carcinoma (ACC). There are only a few LHS cases in the literature. A 68-yearold male presented with obstructive jaundice. ERCP findings showed distal common bile duct (CBD) stricture. Contrast-enhanced CT showed pancreatic head and uncinate process 6X5 Cm hypodense mass. Three weeks after admission, Whipple's procedure and Child's reconstruction were done. H&E and Immuno-histochemistry showed acinar cell carcinoma (ACC). On day three postoperatively the patient developed severe artharitis and inability to walk. Rheumatoid arthritis workup turned to be negative and uric acid level was within normal range. The diagnosis of lipase hypersecretion syndrome (LHS) was made retrospectively. Post-operative lipase hyper-secretion syndrome should be considered in patients with polyarthritis and negative rheumatoid workup in patients with the rare pancreatic acinar cell carcinoma.
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