2485 An Unusual Case of a Primary Mass in the Porta Hepatis

Abstract

INTRODUCTION: Burkitt's Lymphoma is an aggressive subtype of B-cell non- Hodgkin Lymphoma which can present in three forms: endemic, sporadic, and immunodeficiency- associated. It commonly presents in the pediatric population and occurs in less than 1% of adult non-Hodgkin lymphomas. It classically presents with abdominal symptoms with ascites and can commonly involve the distal ileum, stomach, cecum, and mesentery. We describe an unusual case of Burkitt's lymphoma presenting as a mass in the porta hepatis, associated with obstructive jaundice. CASE DESCRIPTION/METHODS: A 74 y/o male presented with a two-week history of jaundice and intermittent epigastric pain. Physical exam findings were scleral icterus, jaundiced skin, and soft nontender abdomen with normal bowel sounds. Labs were pertinent for alkaline phosphatase 1,264, AST 166, ALT 95, total bilirubin 18.8, and direct bilirubin of 15.1. Ultrasound and MRI/MRCP demonstrated a 10.3 × 6.8 × 7.2 mass infiltrating the porta hepatis, peripancreatic, and periduodenal tissues. There were no other masses or adenopathy. At ERCP there was a friable mass replacing the ampulla. Cholangiogram showed dilated intrahepatic ducts proximal to an obstruction at the level of the common bile duct and a metal expandable stent was placed. Biopsy of the ampullary mass revealed high grade, mature B-cell lymphoma. Endoscopic ultrasound revealed a heterogenous mass and fine needle aspiration yielded necrotic cells. Tumor cells were positive for CD20, PAX5, BCL6, CD10, CD20, and LEF1. Pathology was positive for Myc rearrangement and translocation t(8;14). Combination chemotherapy was then initiated. DISCUSSION: Primary tumors of the porta hepatitis are uncommon as it is more often the site of metastatic disease from breast, pancreas, gastric, and colon cancer. This case is particularly unusual in that the primary tumor is a Burkitt lymphoma, which did not arise from lymph nodes or from the pancreas and which presented with obstructive jaundice.