Obstructive jaundice due to hepatobiliary cystadenoma or cystadenocarcinoma

Abstract

Hepatobiliary cystadenomas (HBC) and cystadenocarcinomas are rare cystic lesions. Most patients with these lesions are asymptomatic, but presentation with obstructive jaundice may occur. The first patient presented with intermittent colicky pain and recurrent obstructive jaundice. Imaging studies revealed a polypoid lesion in the left hepatic duct. The second patient had recurrent jaundice and cholangitis. Endoscopic retrograde cholangiopancreatography (ERCP) showed a cystic lesion at the confluence of the hepatic duct. In the third patient with intermittent jaundice and cholangitis, cholangioscopy revealed a papillomatous structure protruding into the left bile duct system. In the fourth patient with obstructive jaundice, CT-scan showed slight dilatation of the intrahepatic bile ducts and dilatation of the common bile duct of 3 cm. ERCP showed filling of a cystic lesion. All patients underwent partial liver resection, revealing HBC in the specimen. In the fifth patient presenting with obstructive jaundice, ultrasound examination showed a hyperechogenic cystic lesion centrally in the liver. The resection specimen revealed a hepatobiliary cystadenocarcinoma. HBC and cystadenocarcinoma may give rise to obstructive jaundice. Evaluation with cross-sectional imaging techniques is useful. ERCP is a useful tool to differentiate extraductal from intraductal obstruction.

Figure 1

Abdominal CT-scan showing a large cystic mass in the left liver lobe with internal septations and calcifications in the cyst wall (A) and ERCP showing a polypoid lesion in the left hepatic duct (arrow) in case 1 (B).

Figure 2

ERCP showing a filling defect due to an intraluminal lesion (arrow) in the left hepatic duct (A), CT-scan showing a cystic lesion with internal septations measuring 3.6 cm located in segment 4 (arrow) (B), specimen after left hemihepatectomy showing macroscopic features of a large lesion (arrow) inside the left bile duct filling up the entire lumen (C), macroscopical cut sections of a multicystic lesion (arrows) encapsulated by a thick fibrous capsule arising from the left hepatic duct (D), microscopical features showing columnar mucinous epithelium with underlying dense-cellular stroma resembling ovarian stroma (arrow) (HE X 200) (E) in case 3.

Figure 3

Abdominal coronal CT-scan showing dilated intrahepatic bile ducts and common bile duct (arrow) (A), ERCP showing filling of a cystic lesion (arrow) connected to the common bile duct, initially diagnosed as a duplicate gallbladder or choledochal cyst (B) in case 4.

Figure 4

Percutaneous transhepatic drainage (PTD) showing complete obstruction at level of the proximal bile duct (A), abdominal CT showing a cystic lesion with irregularly thickened wall in conjunction with dilatated intrahepatic bile ducts (arrow) (B) in case 5.