Management of intrahepatic cholangiocarcinoma in a patient with Caroli's disease: a case report and literature review.

Caroli's disease is a rare congenital disorder characterized by cystic dilatations of the intrahepatic bile ducts, often leading to recurrent bacterial cholangitis, biliary lithiasis, and cirrhosis. In 7%-16% of cases, it progresses to intrahepatic cholangiocarcinoma, emphasizing the need for early diagnosis and intervention. This report presents a 56-year-old patient with recurrent upper abdominal pain, chronic fatigue, and night sweats. Imaging studies (computed tomography, endoscopic retrograde cholangiopancreatography, magnetic resonance cholangiopancreatography) revealed dilated bile ducts and a hypodense lesion in the left hepatic lobe, prompting a left hemihepatectomy. Histopathological analysis confirmed a 12 cm mucinous, papillary cholangiocarcinoma. Postoperative follow-up showed no recurrence or metastasis. Treatment strategies depend on disease extent, with surgical resection being the preferred approach for localized disease, while liver transplantation remains the only curative option for bilateral involvement.