Mucinous Cystic Neoplasms of the Liver and Extrahepatic Biliary Tract

Abstract

Mucinous cystic neoplasms of the liver and extrahepatic biliary tree have recently been re-defined by WHO as epithelial cystic tumours with ovarian-type mesenchymal stroma. Correct recognition of these tumours can be difficult because of their rarity and, conse - quently, lack of awareness by the medical team. Radiological evaluation, including ultra -sonography, computed tomography, magnetic resonance imaging and, upon necessity, positron emission tomography, can yield the correct diagnosis. Radical surgical resection with tumour-free margins is the mainstay of treatment. Adequate treatment approach can be very rewarding, bringing prolonged survival. Here we discuss the up-to-date concepts of definition and classification, theoretical views on tumour origin along with practical issues of clinical presentation, diagnostics, treatment and prognosis. Bile duct compression [10] or invasion can lead to obstructive jaundice and predispose to ascending infection resulting in cholangitis. If the tumour contents are discharged into bile ducts, mucobilia is possible. Bleeding to biliary ways results in haemobilia [ 51 ]. Biliary symp toms are seen in 35% of patients with benign tumours referred to as cystadenomas by WHO classification, 2000 [10] and can be responsible for acute presentation or intermittent course, in addition to the more classical slowly progressing clinical picture.