Intrahepatic mucinous cholangiocarcinoma

Abstract

Mucinous cholangiocarcinoma (MC) is an exceptionally rare variant of intrahepatic cholangiocarcinoma, which comprises a large amount of mucin. 1-4 Here, we report a case of resected MC of the liver. A 56-year-old male patient came into the Emergency Department due to fever and epigastralgia; initial computed tomography (CT) scans revealed a cystic lesion in the S2 segment of liver (Figure 1A) with marked dilated intrahepatic ducts (IHDs) and extrahepatic ducts (EHDs). Abdominal magnetic resonance imaging (MRI) revealed the lobulated lesion, much similar to bile juice or other cystic content, showed prominent hyperintensity on T2-weighted and hypointensity on T1-weighted images. There were no overt enhanced soft tissue components observed on the post-enhanced T1-weighted images. Biliary cystadenoma was impressed at that time, and the primary physician treated the patient with endoscopic retrograde biliary drainage stent placement. However, 16 months later, he came back to our Emergent Department with similar symptoms and signs. Emergent-enhanced CT revealed enlargement of the previously identified multilobulated cyst lesion, which now occupied the entire left hepatic lobe. The lesion F I G U R E 1 Images and pathologic studies of liver tumor. A, Enhanced computed tomography showed a cystic lesion in the left hepatic duct with marked dilated IHDs and EHDs. B, Sixteen months later, enhanced CT showed the development of a poorly enhanced, lobulated cystic tumor with septi in the left hepatic lobe. C, Sixteen months later, MRI showed that the lobulated malignant neoplasm had a high signal on the T2-weighted images in the left hepatic lobe. D, Initial fistulography showed dilated intrahepatic bile ducts of left hepatic lobe. E, Resected liver specimen of the left hepatic lobe. F-G, Microscopically eosinophilic neoplastic cells with abundant mucin production replaced the normal liver parenchyma and were arranged in papillary or tubular structures Hematoxylin and eosing (HE) staining. Original magnification: ×20 and ×200.