Mucobilia: Current aspects in the management of a rare cause of malignant biliary obstruction

Abstract

Mucobilia is a rare pathologic condition characterized by the abnormal secretion and accumulation of abundant mucus within the biliary tree. It is usually seen in association with mucin-producing hepatobiliary and pancreatic tumors. Neoplastic transformation of these tumors can range from low-grade dysplasia to invasive adenocarcinoma. Mucobilia usually presents with signs and symptoms of biliary obstruction, which may span from jaundice to cholangitis with progression to septic complications in severe cases. Occurrence of hepatolithiasis has also been attributed to mucobilia, which raises the concern of an increased risk for the development of cholangiocarcinoma. Precise radiological evaluation and detailed histopathological tissue diagnosis followed by execution of appropriate surgical therapy is vital in the integrated management of mucin-producing biliary neoplasms. This review will address the etiologies and symptomatology of mucobilia as well as discuss current aspects in the management of mucobilia and its causative etiologies. © Japanese Society of Hepato-Biliary-Pancreatic Surgery and Springer 2009.