Neuroendocrine Tumors of Extrahepatic Biliary Tract

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.

Neuroendocrine Tumor Arising from Common Bile Duct: A Case Report and Literature Review

“…Extrahepatic biliary NETs have extremely rare incidence and represent 0. liary NETs were found to be metastasizing into local lymph nodes (19.23%) or the liver (16.7%) [4].…”

“…These tumors involve different organs and sites with a majority of the tumors occurring in pancreas, stomach, small bowel, rectum, and appendix [3]; however, primary NETs of the extrahepatic bile ducts have rare incidence, representing only 0.2% -2% of gastrointestinal NETs [2] [4]. The radiologic findings of biliary NET show well-defined hypervascular solid mass that needs to differentiate biliary cancer and other hypervascular tumors.…”

Non-functioning neuroendocrine tumors of the common hepatic duct: a case report and literature review

“…In grade I (low) and grade II (intermediate), NET are considered well differentiated tumors, as for grade 3 (high), they are called poorly differentiated NEC. [6][7][8] But recently, the new European Neuroendocrine/WHO nomenclature and classification for neuroendocrine tumors has included in addition to the grading system, the mitotic count and the Ki-67 index.…”

“…Preoperative diagnosis was possible in few cases only. 6,7 The prognosis is based on the tumor grading on pathology. They are considered benign when dealing with well to moderately differentiated cells (Grades 1 and 2) and called NET.…”

Surgery in the Era of Molecular Medicine: Review of Gastroenteropanreatic Neuroendocrine Tumours

“…5 Generally, they are non-functioning tumours, although they may express gastrin, serotonin and PP. The high grade BNETs and mixed adenoneuroendocrine carcinomas are more common than the well differentiated (WD) NETs.…”