Challenges in treatment of a patient suffering from neuroendocrine tumor G1 of the hilar bile duct: a case report

Background: Neuroendocrine tumors (NETs) arise from neuroendocrine cells and are extremely rare located in the biliary tract. Currently, there are no guidelines for the diagnosis and treatment of biliary NETs. We present a case with NETs G1 of the hilar bile duct and the challenges for her treatment. Case presentation A 24-year-old woman was presented to our department with painless jaundice and pruritus, and the preoperative diagnosis was the perihilar bile duct cholangiocarcinoma. She underwent Roux-en-Y hepaticojejunostomy with excision of the extrahepatic biliary tree and radical lymphadenectomy. Unexpectedly, postoperative pathology showed a perihilar bile duct NETs G1 and microscopic invasion of the resected right hepatic duct. Then the patient received 3 cycles of adjuvant chemotherapy (Gemcitabine and tegafur-gimeracil-oteracil potassium capsule). At present, this patient has been following up for 20 months without recurrence or disease progression. Conclusions: NETs of the biliary tract are difficult to diagnose preoperatively. The treatment for NETs G1 with R1 resection is still controversial yet may offer potential positive adjuvant chemotherapy. The diagnosis of NETs should be kept in mind by the surgeon for proper time management and more information about biliary NETs should be registered.