Korean J Hepatol.  1999 Jun;5(2):156-161.

A Case of Intrahepatic Cholangiocarcinoma in Polycystic Liver Disease

• Son JH
• Kwon SY
• Song SW
• Yum JH
• Ko JM
• Ahn MS
• Choi KW
• Chang HJ

Abstract

Cholangiocarcinoma has been associated with various fibrocystic diseases of liver and biliary tract, but cholangiocarcinoma in polycystic liver disease (PLD) was extremely rare. It was reported that the prognosis of cholangiocarcinoma associated PLD was very poor and distant metastases were common. We report a rare case of intrahepatic cholangiocarcinoma associated with PLD in 58- year- old female who presented vague abdominal pain. She had also polycystic kidneys with normal renal function. MRI showed well demonstrated tumor with central scar and a adjacent large cyst in the left lobe of the liver. She underwent extended left lobectomy. On microscopic examination, the tumor was moderately differentiated cholangiocarcinoma having abundant fibrous stroma and necrosis and the adjacent cyst showed focal in situ carcinomatous changes in the lining epithelium.

Keyword

Polycystic liver disease; Cholangiocarcinoma; Intrahepatic

MeSH Terms

Abdominal Pain
Biliary Tract
Cholangiocarcinoma*
Cicatrix
Epithelium
Female
Humans
Liver Diseases*
Liver*
Magnetic Resonance Imaging
Necrosis
Neoplasm Metastasis
Polycystic Kidney Diseases
Prognosis
Cholangiocarcinoma