S3481 Silent and Deadly: Cholangiocarcinoma

Abstract

Introduction: Cholangiocarcinoma (CCA) is the most common malignancy of biliary origin. Despite advances in the recognition and treatment of CCA, patient prognosis remains poor. CCA is further sub-classified into intrahepatic CCA (iCCA), perihilar CCA (pCCA) and distal CCA(dCCA). We present a case of pCCA with intrabdominal spread in a patient presenting with abdominal discomfort and weight-loss. Case Description/Methods: A 60-year-old male smoker presented to the emergency department due to one month of intermittent, epigastric abdominal pain. He reported severe colicky pain, associated with nausea and non-bilious, non-bloody emesis. Review of system was notable for anorexia and 20lbs weight-loss. His history was significant for chronic alcohol use disorder and untreated hepatitis C virus. He was hemodynamically stable and appeared cachectic. Epigastric tenderness was present on deep palpation. Initial labs revealed an elevated amylase of 190U/L, and GGT of 237U/L. Total and direct bilirubin were 2.5mg/dL and 0.7mg/dL, respectively. CA19-9, AFP and CEA were negative. Abdominal US revealed a markedly dilated pancreatic duct and a solid, hypoechoic mass within the anterior lobe of the liver. CT demonstrated an irregularly enhancing, lesion measuring 6.3x4.5 centimeters. The lesion appeared to be infiltrative and was encasing the SMA and SMV. CT guided biopsy of the liver mass was obtained. Immunohistochemical staining was CK7+ and CK20+ these findings were highly suggestive of pCCA. Due to intrabdominal spread compromising vasculature the tumor was inoperable, interventional radiology performed transcatheter arterial chemoembolization and radiation. The patient was initiated on chemotherapy with scheduled outpatient follow-up. Discussion: Factors that increase the risk of CCA are excessive alcohol consumption, tobacco smoking and viral infections (hepatitis B & C virus). CT imaging is required for evaluation of tumor spread and histopathological analysis is needed to confirm the diagnosis. Treatment of CCA may include resection, radiation, chemotherapy or transplantation. Due to CCAs lack of response to pharmacological treatment, palliative stenting is often performed for symptomatic relief. Delayed diagnosis of CCA portends a poor prognosis with a median 6-month survival less than 40%. Physicians should focus on improvements in modifiable risk factors, such as cessation of alcohol and tobacco as well as the treatment of HBV & HCV infections. Likewise, treatment outcomes and survival require further attention.Table 1.: Types of Cholangiocarcinoma.