1432 Pancreatic Acinar Cell Carcinoma With Lipase Hypersecretion Syndrome

Abstract

INTRODUCTION: Pancreatic acinar cell carcinoma (PACC) is a rare, highly aggressive, malignant, exocrine-functioning neoplasm. Acinar cells are the most common cells (82%) in the pancreas but accounts for only 1-2% of primary pancreatic neoplasms. In 10-15% of these cases patients can develop lipase hypersecretion paraneoplastic syndrome (LHS) characterized by subcutaneous fat necrosis, polyarthralgia and peripheral eosinophilia. CASE DESCRIPTION/METHODS: A 69-year-old man with history of hepatitis C virus, hypertension, gout, peripheral artery disease and prostate cancer presented with a 1-month history of painful nodules on his bilateral lower extremities (LE). He described progressively worsening joint pain, bilateral LE swelling and purulent drainage from the right LE nodule. Examination revealed hepatomegaly, a 3 × 3 cm nodule on the anteromedial surface of the right LE with purulent drainage, and multiple small, hyperpigmented nodules on bilateral LE. Labs were significant for a white blood cell count of 17.3, hemoglobin of 8.7, normal eosinophil count, albumin of 2.9 g/dL, lipase 5444 IU/L, CA 19-9 44 U/mL and an AFP of 1.8 ng/mL. Computerized tomography revealed an 8.7 × 6.2 × 5.7 cm pancreatic mass encroaching on the 2nd part of the duodenum. Multiple hypoattenuated lesions were seen within the liver largest measuring 4.7 × 5.6 × 7.2 cm with metastatic invasion into the portal vein and superior mesenteric vein. Patient underwent wound debridement. An esophagoduodenoscopy revealed a 5 cm duodenal mass with ulceration extending from the duodenal bulb towards the 2nd portion of the duodenum. Lastly, he underwent an interventional radiology guided liver biopsy. Biopsy of the liver and duodenum both were consistent with PACC. Biopsies from the right leg revealed pancreatic panniculitis. Multi-drug resistant sepsis and recurrent upper gastrointestinal bleeding requiring arterial embolization complicated the hospital course. The patient was placed on comfort care and passed. DISCUSSION: LHS typically presents in advanced disease and is considered a negative prognostic factor. In LHS, PACC overproduces zymogen granules containing lipase, which cause inflammatory and fat necrosis in peripheral tissue through hematogenous spread. Immunochemistry staining is usually positive for trypsin, chymotrypsin and lipase. No standard therapy for PACC has been made but 5-fluorouracil is considered to be effective, and surgical intervention is recommended if possible.