1287 Metastatic Cholangiocarcinoma in a Healthy 27-Year-Old Man: A Case Report

Abstract

INTRODUCTION: Cholangiocarcinoma is a rare, aggressive cancer of the biliary tract most often found in men between the ages of 50 and 70. Risk factors include genetic disorders, such as Lynch syndrome and cystic fibrosis, primary hepatobiliary disease, such as primary sclerosing cholangitis (PSC), and chronic liver disease. This malignancy is highly uncommon in young individuals without PSC. Here we describe the case of a young man with cholangiocarcinoma with recurrent ascites in the setting of a portal vein thrombosis (PVT). CASE DESCRIPTION/METHODS: A 27-year-old man with a history of metastatic cholangiocarcinoma status-post hemihepatectomy with Roux-en-Y hepaticojejunostomy and adjuvant chemoradiation presented with rapidly accumulating ascites secondary to a portal vein thrombosis (PVT) discovered on abdominal imaging. A large-volume paracentesis was performed followed by thrombus recanalization and placement of a portal vein stent. The patient was discharged and presented two weeks later with recurrent ascites and evidence of occlusion of the portal vein stent as well as a new thrombus in the superior mesenteric vein (SMV) and a possible hepatic abscess seen on abdominal imaging. Clinical examination demonstrated a distended abdomen with a positive fluid wave. Laboratory studies revealed elevated white blood cell count (WBC) as well as Klebsiella, Candida, and gram-positive rods (GPR) bacteremia. The benefits versus risks of thrombectomy for recurrent PVT occlusion were considered as though this procedure could ameliorate ascites, the risk of rapid reocclusion was deemed to be high. Furthermore, the patient was at risk for intra- and post-operative complications due to an anticoagulant regimen and unstable clinical status. Ultimately, the patient received repeat LVP and remained on anticoagulants with no additional interventions for recurrent PVT. The patient and his family were discharged home with supportive care. DISCUSSION: Cholangiocarcinoma is a rare, aggressive malignancy of the biliary tract predominantly found in middle-aged men with a high risk of metastasis and a rapidly progressive course. The diagnosis is made on imaging and confirmed on biopsy. Treatment is dependent on disease staging and includes surgery, chemotherapy, and radiation. The disease is rapidly progressive and may predispose patients to pulmonary vein obstruction, leading to portal hypertension and associated sequelae secondary to tumor burden or PVT.