Case Report Patient PP Submitted by:Matthew Clower, MSIV Faculty:Sandra Oldham, MD Date:29 August 2007 Radiological Category:Principal Modality (1): Principal Modality (2): Presented during Radiology Abdominal CT MRI

Case History 80 year-old Caucasian woman presents to gastroenterologist complaining of burning epigastric pain, dysphagia, weight loss, and RLQ pain. PMH of hemicolectomy secondary to diverticulitis, cholecystectomy, and “low- grade hepatitis.” Denies EtOH/Tob/Drugs. Family history of pancreatic and colon cancers. Physical exam was unremarkable and laboratory studies were within normal limits. Endoscopic Gastro-Duodenoscopy (EGD) and abdominal CT were ordered.

Case History On EGD, the patient was found to have a small hiatal hernia. Biopsy of a gastric polyp showed benign histology. The following was found on the abdominal CT:

Radiological Presentations

Hepatocellular Carcinoma Cholangiocarcinoma Hemangioma Lipoma Arteriovenous Malformation Simple Cyst Focal Nodular Hyperplasia Adenoma Transient Hepatic Intensity Difference Which one of the following is your choice for the appropriate diagnosis? After your selection, go to next page. Test Your Diagnosis

5.4 x 4.7 x 4.6 cm mass in the right lobe adjacent to the gallbladder fossa. Associated satellite lesions. Nodular liver with capsular retraction. No involvement of portal venous system or dilation of the bile ducts. Arterial phase enhancement and marked delayed enhancement on CT and MRI. Biopsy showed poorly differentiated carcinoma with occasional gland formation. Hepatocellular Carcinoma Cholangiocarcinoma Hemangioma Adenoma Focal Nodular Hyperplasia Findings: Differentials: Findings and Differentials

Hepatocellular Carcinoma Associated with hepatitis, alcoholism, cirrhosis, and hemochromatosis. Elevated LFTs and decreased synthetic function. MRI: T1 hypointense, T2 hyperintense, intense arterial enhancement. Histology: hepatocyte-like with pseudogland formation. May stain for bile or AFP Cholangiocarcinoma Associated with PSC, liver fluke infection, hepatitis C, cirrhosis, Thorotrast exposure. May present with jaundice or may be asymptomatic. MRI: Homogenous, T1 hypointense, T2 hyperintense, remains enhanced on delayed images. Histology: Typically glandular and well-differentiated, may resemble biliary epithelium Discussion

Hemangioma Asymptomatic and found incidentally. MRI: Nodular enhancement, T1 hypointense, T2 hyperintense. Histology: Reveals vascular structures. Adenoma May rarely cause hepatomegaly and RUQ pain but typically incidentally found. Associated with OCP use. MRI: T1 hyperintense, T2 hyperintense due to fat content. Histology: Uniform hepatocytes. Focal Nodular Hyperplasia Clinically silent. Usually an incidental finding during imaging or autopsy. MRI: Iso/hypointense on T1, iso/hyperintense on T2, central vessels visible, uniform arterial enhancement with delayed Histology: Resembles adenoma. Discussion

Findings most consistent with intrahepatic mass-forming cholangiocarcinoma. Next step: staging to determine resectability, usually with ERCP to evaluate biliary structures and further body imaging to evaluate for metastasis. Diagnosis

Cholangiocarcinoma is a cancer arising from the biliary duct system. Incidence is 1 in 100,000 persons per year in the US (approx 2500 cases/yr). Associated with PSC, liver fluke infection, hepatitis C, cirrhosis, Thorotrast exposure. Tumors are classified by location: intrahepatic (25%), hilar (AKA Klatskin tumor), or extrahepatic. Further classified by morphology: mass-forming, periductal-infiltrating, or intraductal- growing. 90% are adenomatous. Treatment consists of surgical removal or palliative biliary decompression. 5-year survival is 9-18% overall and up to 22-36% for intrahepatic tumors. Diagnosis

Nature Clinical Practice Gastroenterology & Hepatology 2006 AJR 2003

Choi B, Lee J, Han J. Imaging of intrahepatic and hilar cholangiocarcinoma. Abdominal Imaging 2004; 29: Elsayes K, Narra V, et al. Focal Hepatic Lesions: Diagnostic Value of Enhancement Pattern Approach with Contrast-enhanced 3D Gradient-Echo MR Imaging. RadioGraphics 2005;25: Leong T, Leong A. Prognostication in Intrahepatic Cholangiocarcinoma. Adv Anat Pathol 2006;2: Lim J. Cholangiocarcinoma: Morphologic Classification According to Growth Pattern and Imaging Findings. AJR 2003;181: Patel T. Cholangiocarcinoma. Nature Clinical Practice Gastroenterology & Hepatology 2005;1: Emedicine.com. References