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