Intrahepatic Cholangiocarcinoma Arising from Biliary Hamartomas in Patients with Recurrent Acute Cholangitis: A Case Report and Literature Review

Abstract

Biliary hamartomas are tumor-like malformations of the liver. Biliary hamartomas are a type of fibrocystic disorder originating from ductal plate malformation and are typically considered benign, but with the risk of malignant transformation. In this case report, we present a rare occurrence of intrahepatic cholangiocarcinoma (ICC) that developed from biliary hamartomas, along with a literature review. A 76-year-old man with a diagnosis of biliary hamartomas had a history of recurrent cholangitis for 12 years, necessitating cholecystectomy, ERCP, and repeated antibiotic treatments. During his last episode, imaging studies revealed a hypervascular infiltrative mass in the right posterior liver segment. A liver biopsy confirmed adenocarcinoma and subsequent surgical pathology revealed ICC originating from biliary hamartomas. Chronic inflammation in the bile duct associated with biliary hamartomas may serve as a potential trigger for malignant transformation, as observed in this case. Therefore, close surveillance is essential for patients with biliary hamartomas presenting with infectious complications.

Keywords: Biliary hamartoma; Cholangitis; Intrahepatic cholangiocarcinoma.

Fig. 1

(A) An abdominal CT image showing multiple variable-sized, well-defined cystic lesions in the liver. (B) A T2-weighted MR image showing multiple variable-sized, high signal intensity lesions in the liver.

Fig. 2

(A) An abdominal CT image showing ill-defined enhancing lesions in the right posterior segment of the liver (arrow). (B–D) A liver MR image showing an ill-defined arterial enhancing (B) and delayed washout (C, D) lesion in the right liver (arrows).

Fig. 3

(A) A well-demarcated irregular mass is identified (arrow). Its cut surface is pinkish-white, firm, and granular. Multiple nodules are observed in the peritumoral area (arrowheads). (B) Liver parenchyma showing a well-demarcated lesion with small to medium-sized, irregularly shaped dilated glands lined by cuboidal epithelium with intervening fibrous stroma and surrounding inflammatory cells consistent with biliary hamartomas (Hematoxylin and eosin [H&E], ×100). (C) Dysplasia (arrow) adjacent to biliary hamartoma (right lower) is accompanied by chronic inflammation (H&E, ×100). (D) The tumor shows uneven gland distribution and infiltrative borders with tissue destruction (arrow) (H&E, ×40). (E) The tumor forms aberrant glands accompanied by desmoplastic reactions and inflammation (H&E, ×200).