Case Report
Hepatocellular carcinoma in association with bile duct hamartomas: report on 2 cases and review of the literature

https://doi.org/10.1016/j.anndiagpath.2006.12.003Get rights and content

Abstract

Biliary hamartomas are hepatic lesions related to defects in the ductal plate and are part of the spectrum of adult polycystic disease. Microscopically, the lesions are small and consist of irregular bile ducts amidst a fibrous stroma. There are reports on the malignant transformation of biliary hamartomas, particularly on cholangiocarcinoma. Case 1: a 19-year-old woman presented with increased abdominal volume, lumbar pain, jaundice, choluria, cachexia, and progression to hepatic insufficiency. At autopsy, the liver weighed 8850 g and showed a 20.5-cm mass in the right lobe and nodules in the left lobe. Case 2: a 39-year-old man presented with an asymptomatic nodule in the right hepatic lobe. A 6.5-cm liver segment containing a 3.0-cm nodule was excised. Histopathology of both cases revealed hepatocellular carcinoma associated with multiple bile duct hamartomas (von Meyenburg complex). Bile duct hamartoma is generally an incidental finding at laparotomy and autopsy because its course is asymptomatic. The literature has documented its possible progression to malignant neoplasia. However, this relationship has only been established with cholangiocarcinomas. We report herein for the first time on the association of the lesion with hepatocellular carcinoma.

Introduction

Biliary hamartoma is a hepatic lesion, which was first described by von Meyenburg in 1918. It consists of well-defined nodules that are round or irregular in shape and typically show a periportal location. Histologically, the lesion consists of bile ducts amidst collagenized stroma that can be cystic or filled with bile [1]. Biliary hamartomas are mainly incidental findings at laparotomy and autopsy [2]. Malignant transformation has been reported in rare cases, particularly to cholangiocarcinoma [3]. The radiological features of biliary hamartomas are difficult to interpret because of their similarity to hepatic metastases; therefore, the diagnosis is frequently established upon histologic examination [4].

Because there are no reports in the literature regarding the development of hepatocellular carcinoma within the context of bile duct hamartomas, we describe herein 2 cases of this association.

Section snippets

Case 1

A 19-year-old woman presented with a 1-year history of increased abdominal volume, accompanied by lumbar pain, jaundice, choluria and cachexia. Tomography revealed a voluminous hepatic mass with areas of calcification and necrosis. The patient rapidly progressed to hepatic failure and died. Autopsy showed marked hepatomegaly (8850 g) with a 20.5-cm-diameter, extensively necrotic tumoral mass in the right lobe (Fig. 1). In addition, other tumoral nodules and a dilated bile duct were observed in

Discussion

Ductal plate defects are characterized by the persistence or absence of embryonic ductal plate remodeling, which results in congenital disorders of the bile ducts. These disorders include Caroli's disease, autosomal polycystic kidney disease (recessive and dominant variants), congenital hepatic fibrosis, and bile duct hamartomas, also known as von Meyenburg complexes. The clinical presentation and evolution of these disorders vary, ranging from neonatal death (eg, in cases of the infantile,

Acknowledgment

The authors thank Ms Maria C. Aparecida Nascimento for the secretarial assistance.

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