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Non-functioning well-differentiated neuroendocrine tumor of the extrahepatic bile duct: an unusual suspect? |
Harsheet Sethi, Mansoor Madanur, Parthi Srinivasan, Bernard Portmann, Nigel Heaton and Mohamed Rela |
London, UK
Author Affiliations: Department of Hepatobiliary and Transplant Surgery (Sethi H, Madanur M, Srinivasan P, Heaton N and Rela M); and Department of Histopathology (Portmann B), Kings College Hospital, Denmark Hill, London SE5 9RS, UK
Corresponding Author: Mohamed Rela, FRCS, Institute of Liver Studies, Kings College Hospital, Denmark Hill, London SE5 9RS, UK (Tel: 0044-2032999000ext4801; Fax: 0044-2073463575; Email: Mohamed.rela@kcl.ac.uk) |
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Abstract BACKGROUND: Neuroendocrine tumors (NETs) arising in the biliary tree are extremely rare, and 37 cases were identified in the English literature.
METHODS: A well-differentiated NET was found arising from the junction of the cystic and common hepatic ducts, in a 51-year-old male presenting with pedal edema and weight loss with abnormal liver enzymes and a normal serum bilirubin level. No mass was seen on radiological imaging and biopsy of the liver was suggestive of an early cholangiopathy. A bile leak complicating the liver biopsy led to an ERCP that demonstrated a filling defect suggestive of a mass in the common bile duct (CBD).
RESULTS: He underwent a successful excision of the tumor with a Roux-en-Y hepaticojejunostomy. The diagnosis of NET was made on histological and immunohistochemical analysis of the resected specimen. He remains well and disease free 22 months after surgery.
CONCLUSIONS: Recognition of biliary NET continues to be a challenge and an increased awareness of these tumors in rare sites will result in optimal management of these tumors.
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