Abstract

INTRODUCTION: Cholangiocarcinoma (CC) is the most common malign epithelial tumor of biliary tract and is the most common primary liver malignancy following hepatocellular carcinoma (1). 50% of CC is perihilar, 40% is distal and less than 10% is intrahepatic located (2).Its incidence is quite different based on geographical regions and ethnical background. The highest incidence was found in Southern Asia (113/100000) and the lowest incidence was found in Australia (0.1/100000) (3). Five years survival rates are low (22-42%) even in intrahepatic cholangiocarcinoma (ICC) patient who had resection (4). We reported a badly prognosed ICC case in a young adult without a risk factor for cholangiocarcinoma.

CASE: A 24-year-old male patient referred to us with jaundice and blunt abdominal pain which started two weeks ago and spread to right hypochondrium, right lumbar and epigastric area. On physical examination the skin and scleras were icteric. Hepatomegaly and tenderness was detected at the right upper quadrant. The laboratory findings were given in Table-1. Hepatomegaly, splenomegaly, mild ascites and mass lesions that reaches 6 cm at diameter and propensity to coalesce on the right lobe of the liver was observed at abdominal US. In contrast-enhanced diffusion MRI, a 114x62 mm mass lesion which was heterogeneously hyperintense according to T2A sequence and iso-hyperintense according to T1A sequence was observed in the right lobe of the liver in segment 6-7. Ultrasound guided tru-cut biopsy was made on the mass in the liver. Glandular structured, medium degree atypia-containing tumoral cells also infiltrating the liver parenchyma in fibrous stroma were observed in histopathological examination. CK7, CK19, EMA and MOC 31 were detected positive in tumor cells in immunohistochemical analysis [Figure-1]. ICC diagnose was set by clinical and histopathologic findings. There were malignant mass lesions that spread almost all segments of the liver, abdominal hypermetabolic ascites and lymphadenopathy at positron emission tomography Chemotherapy was not planned due to advanced stage of disease, widespread metastasis and bad patient performance. He had asterixis and was confused on the 12th day of his admission to the clinic. The patient was exitus due to acute hepatic failure.

CONCLUSION: Cholangiocarcinoma constitutes 3% of all gastrointestinal malignities (5). Average diagnosis age is over 50 years of age globally and is around 65 years of age in western societies (3). CC is seen rarely under 40 years of age when CC occurring in primary sclerosing cholangitis background is excluded (6). Our patient had no CC risk factors and wasn’t in the age group it is seen frequently. The patient was exitus due to acute hepatic failure in a month’s time. Median survival is less than 2 years in unresectable patients (7). ICC generally seen in middle aged or elderly patients can also be seen in young adults and its mortality is quite high.