3017 Cholangiocarcinoma: A Formidable Diagnosis

Abstract

INTRODUCTION: Cholangiocarcinoma is a malignant epithelial cancer of the biliary tract. The disease is difficult to diagnose and is usually fatal due to its late clinical presentation, lack of effective non-operative therapy, and rapid turnover. The typical age at presentation is the seventh decade. We describe a case of unanticipated cholangiocarcinoma in a 32 year old male. CASE DESCRIPTION/METHODS: A 32 year old male, with history of childhood Wilm's tumor status post resection, presented with left lower quadrant abdominal pain and 20 pound weight loss over one month. The pain was described as LLQ pressure radiating to the midline and across the abdomen. In addition he reported nausea without emesis, significant abdominal distention causing dyspnea, three episodes of possible GI bleeding (including two episodes of melena and one of hematochezia), light stools and dark urine. CT revealed metastatic disease with diffuse nodal involvement and biopsy showed adenocarcinoma of unknown origin. The diagnosis was originally considered a recurrence of Wilm's tumor but repeat biopsy with IHC revealed primary cholangiocarcinoma. The patient underwent paracentesis and was started on Gemcitabine and Oxaliplatin chemotherapy. Restaging CT showed progression of disease with extensive tumor thrombus extending from the hepatic vein into the IVC and right heart, bilateral pleural effusions, innumerable pulmonary nodules and vertebral metastasis. Due to poor functional status he was not a candidate for further chemotherapy or immunotherapy. Symptomatic treatment was initiated but his condition rapidly declined and he eventually succumbed. DISCUSSION: Cholangiocarcinoma is the second most common primary malignant tumor in the liver. It can be intrahepatic or extrahepatic. Patients with extrahepatic tumors usually present with painless jaundice from biliary obstruction, whereas intrahepatic tumors usually present with pain. Common complaints include pruritus, abdominal pain, weight loss, fever, and symptoms of biliary obstruction such as clay-colored stools and dark urine. Contrast CT is sensitive for detecting intrahepatic tumors, the level of biliary obstruction, and also permits visualization of the pertinent nodal basins. Cholangiocarcinoma presents a formidable diagnostic and treatment challenge. The majority of patients present with unresectable disease and have a survival of less than 12 months following diagnosis.