Neuroendocrine Carcinoma of the Extrahepatic Bile Ducts: A Case Report

Denis Jevdokimov (Coresponding Author), Elina Tauvena, Natalija Jevdokimova, Sofija Vilisova, Olga Kriviča, Inese Briede, Tatjana Tone, Aiga Staka, Aija Gerina-Berzina, Arturs Ozolins, Aldis Pukitis

Research output: Contribution to journalArticlepeer-review


Patient: Final Diagnosis: Symptoms: Clinical Procedure: Specialty: Objective: Background: Case Report: Conclusions: Female, 42-year-old Biliary neuroendocrine carcinoma involving the common hepatic duct • common bile duct and cystic duct PT1N0M0G3R0 Itching of the hands, forearms and lower legs, followed by symptoms of obstructive jaundice Cholecystectomy • ERCP • resection of a hepatobiliary mass Gastroenterology and Hepatology • Oncology • Pathology • Surgery Rare disease Neuroendocrine tumors (NETs) primarily originating from the extrahepatic biliary (EB) tree are a medical rarity, accounting for less than 100 recorded instances globally. This case report outlines an encounter with this un-common condition, demonstrating the complexities of diagnosis and management. A 42-year-old woman presented at our Emergency Department with a 3-week history of itching and symptoms of obstructive jaundice. Initial laboratory tests showed hyperbilirubinemia and elevated liver transami-nases. Abdominal ultrasonography indicated choledocholithiasis. Magnetic resonance imaging suggested ei-ther Mirizzi syndrome or a proximal common bile duct neoplasm. Abdominal computed tomography showed cholestasis, suggesting choledocholithiasis or cholangiocarcinoma (type-1). An endoscopic retrograde cholan-giopancreatography with biliary and pancreatic duct stenting was performed for drainage, with brush cytolo-gy confirming adenocarcinoma. The patient was referred for surgical resection of the bile duct tumor, involving extrahepatic bile duct resection, en bloc cholecystectomy, lymphadenectomy, Roux-en-Y anastomosis, and biliary drainage. Histopathology identified a neuroendocrine carcinoma. Following surgery, the patient under-went eight cycles of FOLFOX6 chemotherapy, with no disease relapse post-treatment. This case emphasizes multidisciplinary teamwork importance in managing rare diseases like EB bile duct NETs. These tumors’ rarity and symptom ambiguity necessitate histological examination for accurate diagnosis. This report aims to guide healthcare professionals facing similar future cases.

Original languageEnglish
Article numbere939239
JournalThe American journal of case reports
Publication statusPublished - 21 Jun 2023


  • Female
  • Humans
  • Adult
  • Choledocholithiasis/surgery
  • Neoplasm Recurrence, Local
  • Bile Ducts, Extrahepatic/pathology
  • Carcinoma, Neuroendocrine/diagnosis
  • Bile Duct Neoplasms/diagnosis
  • Bile Ducts, Intrahepatic/pathology

Field of Science*

  • 3.2 Clinical medicine

Publication Type*

  • 1.1. Scientific article indexed in Web of Science and/or Scopus database


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