Neuroendocrine Tumor of the Common Bile Duct: Case Report

Abstract

Carcinoma of the extrahepatic biliary tract accounts for <2% of all cancers. Neuroendocrine tumor of the extrahepatic bile duct is very rare, and there are <200 cases reported since 1959. The preoperative diagnosis is infrequent (5.12%). The definite diagnosis relies on postoperative pathology which utilized immunohistochemistry study on many biomarkers to diagnose the histological subtypes of neuroendocrine neoplasms, such as chromogranin A, synaptophysin, and neuron-specific enolase. When the primary tumor has no metastases, radical removal of the lesion appears as curative treatment. The treatment of the carcinoid syndrome or other functioning syndrome is the first priority. We report a case of a 12-year-old Mexican woman with neuroendocrine tumor of the extrahepatic bile duct (common bile duct neuroendocrine tumor) seen in our hospital. Resection of the common bile duct, cholecystectomy, end to side Roux-en-y hepaticojejunostomy, and portal lymphadenectomy was performed. A review of the pertinent literature was performed. Given the rarity of the disease, treatment principles are based mainly on retrospective series and case reports. We present the eighth case in adolescence in the literature.

Neuroendocrine Carcinoma of the Extrahepatic Bile Ducts: A Case Report

“…However, according to data from literature, those arising in the gastrointestinal tract and the pancreas are extremely rare, accounting for approximately 1% to 1.5% of all gastroenteropancreatic neoplasms. The annual age-adjusted incidence of gastroenteropancreatic neuroendocrine neoplasms in the United States was 3.56 per 100 000 persons in 2012, which is rare, but steadily increasing [ 5 , 6 ]. The most common origin from the digestive system was the small intestine, which was approximately 1.05 per 100 000 persons, then the rectum (1.04 per 100 000 persons), and only then the pancreas (0.84 per 100 000 persons).…”

“…Statistical resources mentioned that 0.32% of neuroendocrine neoplasms occur in the extrahepatic bile duct, and almost all of them are well-differentiated NETs. Poorly differentiated neuroendocrine carcinomas of the extrahepatic bile duct are rare, reportedly accounting for only 0.19% of extrahepatic bile duct malignancies [ 5 , 6 ]. The most frequent sites of extrahepatic biliary NETs are the common hepatic duct and distal common bile duct (19.2%), followed by the middle of the common bile duct (17.9%), cystic duct (16.7%), and proximal common bile duct (11.5%) [ 7 ].…”

Carcinoma Neuroendócrino De Pequenas Células Pouco Diferenciado Em Colédoco Distal: Um Relato De Caso

“…A segunda revisão foi publicada por Z. Liu em 2018, a qual restringiu sua busca apenas aos casos de TNE no colédoco, acrescentando 8 casos aos 38 já revisados 7 . Até 2021, havia menos de 200 casos reportados de todos TNVBE no mundo inteiro 8 .…”

Neuroendocrine carcinoma of the common hepatic duct coexisting with distal cholangiocarcinoma: A case report and review of literature

“…The cause of EBNEC remains unclear. Chronic inflammation of the bile duct can lead to metaplasia of endocrine cells scattered on the bile duct epithelium, which may be the initial stage of NEC development[ 3 ].…”