Mucobilia in Association With a BiliaryCystadenocarcinoma of the Caudate Duct:A Rare Cause of Malignant Biliary Obstruction

Abstract

Mucobilia is a rare condition characterized by the accumulation of abundant mucus within the intra-or extrahepatic biliary tree. A variety of hepatobiliary and pancreatic neoplasms are mucin producing and have been associated with the development of mucobilia including biliary mucinosis, biliary papillomatosis, mucin-producing cholangiocarcinoma (MPCC), or cystic neoplasms of the pancreas or biliary tree (cystadenoma or cystadenocarcinoma). We report the case of 46 year-old male with a biliary cystadenocarcinoma of the caudate lobe which resulted in chronic biliary obstruction and relapsing cholangitis. A review of the literature for both mucobilia and biliary cystadenocarcinoma is provided along with a discussion addressing the clinical presentation, diagnosis, treatment, and prognosis for this rare entity.

Invasive biliary mucinous cystic neoplasm: a review

“…Although there are no specific markers or characteristics that can consistently identify intrahepatic BMCNs, standard liver cancer markers should be considered to rule out other similarly presenting tumours (see Table 1). Liver function tests are generally normal, but elevated levels of bilirubin, alkaline phosphatase and gamma‐glutamyl transpeptidase (GGT) have been seen in cases in which intra‐ or extrahepatic biliary duct compression are present 4,56,57 . CA 19‐9 may be elevated (particularly if there is an associated invasive carcinoma), but CEA and alpha‐fetoprotein (AFP) are usually normal 19,58…”

“…It is particularly difficult to evaluate because distant metastasis is seen to occur very infrequently as local invasion and intrahepatic recurrence after excision tend to be the primary modes of malignant behaviour 4,22 . Distant metastases have been reported to occur in up to 20% of patients and up to 13% have been noted to have lymphatic spread 2,57 . The most common sites of metastasis are the lungs, pleura, peritoneum, liver, duodenum, stomach and pancreas 1,79 .…”

“…This is because primary biliary invasive MCNs appear to have a less invasive nature and a slower growth rate than other malignancies of the liver and invasive MCNs of other sites, such as the pancreas or ovaries 22,79 . Overall 5‐year survival after resection is 65−71% and can reach 100% in patients in whom histologically negative resection margins are achieved 31,57,79 …”

Asymptomatic hepatobiliary cystadenoma of the hepatic caudate lobe: a case report

“…There are only two reported cases of hepatobiliary cystadenoma originating from the caudate lobe, and both cases were symptomatic[2],[3]. It has been reported that hepatobiliary cystadenoma prolapses into the extrahepatic bile duct[6].…”

Intraductal mucinous tumors occurring simultaneously in the liver and pancreas

“…Indeed, there have been some reports describing such cases in patients with imaging abnormalities or macroscopic findings similar to those of intraductal mucinous liver tumors. 12,13 However, it is impossible to discuss these cases here, because of insufficient pathological descriptions, in particular, insufficient descriptions of the intratumor interstitium. In 1991, Kawarada et al 14 classified cystic malignant tumors of the liver into the following three types, based on their developmental origins and morphological characteristics: cystic adenocarcinoma (group A), bile duct carcinoma with intrahepatic bile duct dilatation (group B), and degeneration cyst in malignant liver tumors (group C).…”