Introduction and importance Biliary tree neuroendocrine tumors (NET) are rare, with 100 cases in the literature, and have an excellent prognosis. Although they are rarely diagnosed before surgery, complete surgical excision offers optimal treatment. Case presentation We report a case of a 36-year-old female patient referred to the surgical team with obstructive jaundice for which she was investigated and found to have a common bile duct tumor showing proximal obstruction. Excision of the tumor with hepaticojejunostomy was done. Later on, the pathology report showed grade-1, well-differentiated neuroendocrine carcinoma, which was completely excised. No further intervention was provided to the patient. Clinical discussion Complete surgical resection with excision of the extrahepatic bile ducts and portal lymphadenectomy in addition to Roux-en-Y hepaticojejunostomy or even pancreaticoduodenectomy for distal CBD neuroendocrine tumors gives sufficient treatment in the majority of cases. No evidence of the advantage of chemo-radiotherapy as part of the treatment for this tumor. Conclusion Biliary tree neuroendocrine tumors are benign tumors, and it is usually difficult to ascertain the diagnosis preoperatively. However, complete surgical excision offers an optimal treatment with no evidence of chemotherapy or radiotherapy's role in the management.
Highlights • Biliary tree NETs have an excellent prognosis with a 10-year survival of 80% • complete surgical excision offers optimal treatment for biliary tree NET • Size of the tumor, presence of lymphovascular invasion, and Ki-67 assesses prognosis