Case Report
A rare cause of recurrent cholangitis: neuroendocrine tumour of the main bile duct: case report and review of literature
Abstract
Background: Neuro-endocrine neoplasms (NEN’s), formerly called “carcinoids”, appear to be a heterogeneous group of tumours varying in location, size and clinical symptoms. Given the difference in functional and biological behaviour, the World Health Organization (WHO) established criteria to classify NEN’s irrespective of their site of origin, which could predict the biological behaviour with high probability.
Methods: We present the case of a 75-year-old female patient with recurrent cholangitis, itching and limited weight loss. After diagnostic work up, a solid mass obstructing the common bile duct (CBD) was found. Since no distant metastases were seen, resection was decided upon with hepaticojejunostomy and Roux-en Y reconstruction. The pathology report showed a neuro-endocrine neoplasm (NEN) of the extrahepatic bile duct (EHBD).
Results: NEN’s are an exceptional cause of conjugated hyperbilirubinemia in the presence of dilated intra- and extra-hepatic bile ducts. If one suspects an intrinsic tumour of the extra-hepatic bile duct, a cholangiocarcinoma accounts for 80-90%. NEN’s of the EHBD appear to have a more indolent biologic evolution than cholangiocarcinomas. However, a preoperative differentiation is often difficult, as NEN’s are located in the submucosa and endoscopic brush cytology has a low sensitivity.
Conclusions: Based on the current knowledge (142 case reports) concerning NEN’s of the EHBD, grade is more important than size for prognosis and R0 resection remains the cornerstone for long time survival, even in a metastasized setting.
Methods: We present the case of a 75-year-old female patient with recurrent cholangitis, itching and limited weight loss. After diagnostic work up, a solid mass obstructing the common bile duct (CBD) was found. Since no distant metastases were seen, resection was decided upon with hepaticojejunostomy and Roux-en Y reconstruction. The pathology report showed a neuro-endocrine neoplasm (NEN) of the extrahepatic bile duct (EHBD).
Results: NEN’s are an exceptional cause of conjugated hyperbilirubinemia in the presence of dilated intra- and extra-hepatic bile ducts. If one suspects an intrinsic tumour of the extra-hepatic bile duct, a cholangiocarcinoma accounts for 80-90%. NEN’s of the EHBD appear to have a more indolent biologic evolution than cholangiocarcinomas. However, a preoperative differentiation is often difficult, as NEN’s are located in the submucosa and endoscopic brush cytology has a low sensitivity.
Conclusions: Based on the current knowledge (142 case reports) concerning NEN’s of the EHBD, grade is more important than size for prognosis and R0 resection remains the cornerstone for long time survival, even in a metastasized setting.
