C ASE R EPORT: Mucinous cholangiocarcinoma featuring a multicystic appearance and periportal collar in imaging

Abstract

A case of mucinous cholangiocarcinoma (CC), a rare histological type of CC, featuring unusual images is reported. The patient was hospitalized because of acute development of jaundice and fever. Computed tomography demonstrated multiple cystic lesions in the liver and a band-like low density area parallel to the intrahepatic portal vein, a so-called 'periportal collar'. Endoscopic cholangiography revealed a stricture of the hepatic duct with slight upstream dilatation. Cytology of the bile juice and fine-needle aspiration of the cystic lesion in the liver disclosed mucinous carcinoma. The patient died of multiorgan failure 3 weeks after admission. The autopsied liver showed that multiple mucus lakes were lined with adenocarcinoma cells and signet ring cells were floating in the mucus lakes. The cancer cells had spread along the portal tract and invaded into the hepatic parenchyma.

Mucinous cholangiocarcinoma: Clinicopathological features of the rarest type of cholangiocarcinoma

“…Mucinous cholangiocarcinoma is the rarest type of cholangiocellular carcinoma,12, 20 and this carcinoma is reported to be derived from IPNB 15, 16, 21. IPNB is pathologically characterized by prominent intraductal papillary proliferation with delicate fibrovascular cores and frequent mucin hypersecretion,15, 16, 21 and it is regarded as the biliary counterpart of intraductal papillary mucinous neoplasm of the pancreas.…”

Colloid Carcinoma of the Extrahepatic Biliary Tract with Metastatic Lymphadenopathy Mimicking Cystic Neoplasm: A Case Report

“…In the digestive tract, it is relatively common in the stomach and colon with poor prognosis, while infrequent in the pancreas with better prognosis than ductal adenocarcinoma (5). It is very rarely seen in the biliary tract with a 0.8% reported incidence in autopsied cholangiocarcinoma cases as described in ‘mucinous intrahepatic cholangiocarcinoma’ by Nakajima et al in 1988 (4). The clinical and imaging features of colloid carcinoma of the EHD have not yet been described due to rarity of this subtype.…”

“…Hayashi et al described radiologic characteristics of two cases of mucinous type intrahepatic cholangiocellular carcinoma, including large mucinous lakes throughout the tumor without mucin excretion into the bile duct (3, 9). Mizukami et al (4) reported a unique presentation of mucinous carcinoma, most likely originating from the right hepatic duct. CT demonstrated multiple cystic lesions distributed adjacent to the portal area with a beaded appearance.…”

“…CT demonstrated multiple cystic lesions distributed adjacent to the portal area with a beaded appearance. This so called ‘periportal collar,’ reflects the tendency to form mucus lakes along the portal area (4). Despite similar histological features, differences in imaging findings between studies may be due to differences in the tumor origin and the mode of spread (4).…”

“…While several previous reports have described ‘mucinous cholangiocellular carcinoma of the liver’ or ‘mucin producing bile duct tumor arising from the intrahepatic duct’, there have been no reports of colloid carcinoma of the EHD in the radiologic literature (3, 4). Colloid carcinomas are different from other mucin hypersecreting tumors such as intraductal papillary neoplasms, mucinous cystic neoplasms of the biliary tract, and ductal adenocarcinomas.…”