Intrahepatic cholangiocarcinoma in a patient with Wilson's disease: a case report

Abstract

The incidence of hepatobiliary malignancies, and especially intrahepatic cholangiocarcinoma (ICC), for patients with Wilson's disease (WD), is very low, even for cirrhotic patients. A 44-year-old male was admitted to our department for treatment of a liver tumor. He was diagnosed with WD at the age of 15. According to radiological findings, his liver tumor was a suspected hepatocellular carcinoma (HCC) or a combined hepatocellular and cholangiocellular carcinoma. A partial resection of liver segments 8 (S8) and 5 (S5) was subsequently performed due to the intraoperative suspicion of intrahepatic metastasis at the surface of S5. Postoperative histology revealed that the resected portion of S8 contained an ICC; the removed S5 portion comprised a regenerative nodule with hemosiderosis. To date, the patient has survived without tumor recurrence for more than 44 months following surgery. A survey of the literature, inclusive of case reports, would suggest that surgical resection is the primary course of action for a WD patient with ICC, if liver function can be preserved and curative resection performed.

Keywords: Hepatobiliary malignancies; Intrahepatic cholangiocarcinoma; Liver tumor; Wilson’s disease.

Fig. 1

Preoperative radiological findings. a Abdominal ultrasonography showed a hypoechoic lesion in segment 8 (S8) of the liver. b The main tumor (S8) showed an early enhancement in CT, which was prolonged until the delayed phase (c). d Gadolinium ethoxybenzyl diethylenetriaminepentaacetic acid (Gd-EOB-EDTA)-enhanced MRI revealed a solitary 1.8-cm tumor, with a low-intensity signal in the hepatobiliary phase in the transverse (d) and sagittal planes (e)

Fig. 2

The resected specimens of S8 (a) and S5 (b). Histological examination of the resected specimens. c The lesion in S8 was a moderately differentiated cholangiocarcinoma of the liver (HE staining, ×100). The adjacent, non-cancerous liver parenchyma demonstrated typical cirrhotic features with steatosis (HE staining, ×40) (d), (HE staining, ×100) (e)