Hepatobiliary and Pancreatic: Biliary hamartoma manifests as liver cysts
Journal
Journal of Gastroenterology and Hepatology (Australia)
Journal Volume
35
Journal Issue
1
Pages
11
Date Issued
2020
Author(s)
Abstract
A 79 year old man with chronic hepatitis C related cirrhosis found to have multiple ill-defined liver lesions on abdominal ultrasound. Infiltrative hepatocellular carcinoma was suspected. He was then referred to our hospital for further evaluation. Apart from fatigue in the recent weeks, the patient did not have any abdominal pain, weight loss or change in appetite. Physical examination revealed a distented abdomen with hepato-splenomegaly. The laboratory investigations showed normal liver function tests and alpha-fetoprotein level. Because of poor renal function, non-contrast magnetic resonance imaging (MRI) was arranged. The image study revealed numerous hepatic cystic lesions in both lobes of the liver, which were hypointense on T1-weighted images and hyperintense on T2-weighted images (Fig. 1a&b). Magnetic resonance cholangiopancreatography (MRCP) showed no communication between hepatic cystic lesions and biliary trees (Fig. 1a&b). Biliary hamartoma was suspected, but diffuse type hepatocellular carcinoma or multiple liver metastases could not be excluded. As a result, the patient underwent liver biopsy for tissue proof, showing clusters of dilated bile ductules with angulation and few intraluminal bile material in portal area, which revealed a variable degree of dense fibrous or hyalinized stroma (Fig. 1c). The ducts were lined by cuboidal or occasionally flattened epithelium without nuclear atypia or mitotic activity (Fig. 1d). These findings were compatible with biliary hamartoma, or also known as Von Meyenburg complex. Biliary hamartoma is a rare disease which results from ductal plate malformations of the smallest intrahepatic bile ducts due to disordered embryonic involution. It is usually asymptomatic and to be found incidentally as this case, although some case series reported non-specific abdominal discomforts and became an infectious complication involving the liver parenchyma. The computed tomography appearance of biliary hamartoma consists of multiple, irregular, and small hypoattenuated areas, which do not normally enhance on contrast injection. MRI with MRCP is the best tool for the diagnosis. In contrast to normal liver parenchyma, biliary hamartoma is usually presented as diffuse tiny or small cystic lesions in the liver without enhancement. The cystic lesions are usually less than 1.2 cm in size and not communicating with bile ducts. The cystic lesions of Caroli's disease are dilated intrahepatic ducts. The fungal microabscesses usually have fever and wall enhancement in immunocompromised patients. Because malignant transformation of biliary hamartoma has been described, particularly cholangiocarcinoma, periodical follow-up should be considered.
Other Subjects
abdominal distension; abdominal radiography; aged; Article; biliary tract tumor; case report; chronic hepatitis C; clinical article; echography; fatigue; hamartoma; hepatosplenomegaly; human; human cell; human tissue; kidney function; liver biopsy; liver cirrhosis; liver cyst; male; nuclear magnetic resonance imaging; priority journal; biliary tract disease; complication; cyst; diagnostic imaging; differential diagnosis; diffusion weighted imaging; hamartoma; liver disease; magnetic resonance cholangiopancreatography; pathology; Aged; Biliary Tract Diseases; Cholangiopancreatography, Magnetic Resonance; Cysts; Diagnosis, Differential; Diffusion Magnetic Resonance Imaging; Hamartoma; Humans; Liver Diseases; Male
Type
journal article