Neuroendocrine Tumors of Extrahepatic Biliary Tract

Nickos Michalopoulos; Theodossis S. Papavramidis; Georgia Karayannopoulou; Ioannis Pliakos; Spiros T. Papavramidis; Ioannis Kanellos

doi:10.1007/s12253-014-9808-4

Pathology & Oncology Research

October 2014, Volume 20, Issue 4, pp 765–775

Neuroendocrine Tumors of Extrahepatic Biliary Tract

Authors
Authors and affiliations

Nickos MichalopoulosEmail author
Theodossis S. Papavramidis
Georgia Karayannopoulou
Ioannis Pliakos
Spiros T. Papavramidis
Ioannis Kanellos

Review

First Online:: 11 June 2014

Received:: 14 September 2013
Accepted:: 28 May 2014

DOI: 10.1007/s12253-014-9808-4

Cite this article as:: Michalopoulos, N., Papavramidis, T.S., Karayannopoulou, G. et al. Pathol. Oncol. Res. (2014) 20: 765. doi:10.1007/s12253-014-9808-4

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 up-to-date. One hundred articles, describing 150 cases were collected. Each article was carefully analyzed and a database was created. The most common symptoms were jaundice (60.3 %) and pruritus (19.2 %). Cholelithiasis co-existed in 15 cases (19.2 %). 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 (19.2 %). Surgical management was considered the main treatment for EBNETs, while excision of extrahepatic biliary tree (62.82 %) with portal vein lymphadenectomy (43.6 %) 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.

Keywords

Neuroendocrine tumor Carcinoid Biliary neoplasm Klatskin tumor Extrahepatic biliary duct obstruction

Abbreviations

NET: Neuroendocrine tumor
EBNET: Extrahepatic biliary neuroendocrine tumor
WHO: Word health organization
APUD: Amine precursor uptake and decarboxylation
SD: Standard deviation
NSE: Neuron-specific enolase
ERCP: Endoscopic retrograde cholangiopancreatography
VIP: Vasoactive intestinal peptide
CT: Computed tomography
MRI: Magnetic resonance image
VHL: Von Hippel-Lindau syndrome
5-HIAA: 5-hydroxyindoleacetic acid
ZES: Zollinger ellison syndrome
PTC: Percutaneous transhepatic cholangiography
G: Grade
HPF: High power fields

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Authors and Affiliations

Nickos Michalopoulos
- 1
Email author
Theodossis S. Papavramidis
- 1
Georgia Karayannopoulou
- 2
Ioannis Pliakos
- 1
Spiros T. Papavramidis
- 1
Ioannis Kanellos
- 1

1.3rd Department of Surgery, AHEPA University HospitalAristotle University of ThessalonikiThessalonikiGreece
2.Pathology Department, AHEPA University HospitalAristotle University of ThessalonikiThessalonikiGreece

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Neuroendocrine Tumors of Extrahepatic Biliary Tract

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