Authors: Nickos Michalopoulos Theodossis S Papavramidis Georgia Karayannopoulou Ioannis Pliakos Spiros T Papavramidis Ioannis Kanellos
Publish Date: 2014/06/11
Volume: 20, Issue: 4, Pages: 765-775
Abstract
Neuroendocrine tumors of the extrahepatic bile ducts EBNETs are very rare The aim of the present review is to elucidate the characteristics of EBNETs their treatment and prognosis An exhaustive systematic review of the literature was performed from 1959 uptodate One hundred articles describing 150 cases were collected Each article was carefully analyzed and a database was created The most common symptoms were jaundice 603 and pruritus 192 Cholelithiasis coexisted in 15 cases 192 Hormone and vasoactive peptide related symptoms were present in only 7 cases 9 The most frequent sites were found to be the common hepatic duct and the proximal common bile duct 192 Surgical management was considered the main treatment for EBNETs while excision of extrahepatic biliary tree 6282 with portal vein lymphadenectomy 436 was the most popular procedure EBNETs are extremely rare Their rarity makes their characterization particularly difficult Up to date the final diagnosis is made after surgery by pathology and immunohistochemistry findings The present analysis of the existing published cases elucidates many aspects of these tumours giving complete clinicopathological documentation
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