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Primary neuroendocrine tumour of the extrahepatic biliary duct
  1. Sarah Fennelly1,2,
  2. Basilie Teoh3,
  3. Jyothirmayi Velaga4 and
  4. Nezor Houli3
  1. 1General Surgery, Northern Hospital Epping, Epping, Victoria, Australia
  2. 2Master of Cancer Sciences, The University of Melbourne Faculty of Medicine Dentistry and Health Sciences, Melbourne, Victoria, Australia
  3. 3Hepatobiliary Surgery, Northern Hospital Epping, Epping, Victoria, Australia
  4. 4Radiology, Northern Hospital Epping, Epping, Victoria, Australia
  1. Correspondence to Dr Sarah Fennelly; sarah.fennelly{at}gmail.com

Abstract

Neuroendocrine tumours of the extrahepatic bile ducts are extremely rare, accounting for only 0.2–2% of gastrointestinal tract neuroendocrine neoplasms. Here, we present an incidental finding of this rare tumour, identified during staging scans post-resection of a malignant rectosigmoid polyp and treated with resection and biliary reconstruction. A suspicious, arterially enhancing porta hepatis lymph node was identified on CT and further investigated with liver MRI and 68Ga DOTATATE PET. These revealed a lobulated lesion of the hilum which showed marked avidity without metastases. The patient underwent a successful radical resection of the extrahepatic biliary ducts (including the left, right and common hepatic ducts), the common bile duct and hepaticojejunostomy formation. Histology revealed a 20-mm well-differentiated NET, WHO Grade 2. Two years later, the patient is alive and well without recurrence. This fascinating case illustrates the importance of thorough preoperative planning of surgical resection for tumours at an unusual primary site.

  • Anatomic variation
  • Biliary intervention
  • Cancer intervention
  • Common bile duct
  • Hepatic cancer

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Footnotes

  • SF and BT are joint first authors.

  • Contributors The following authors were responsible for drafting the text, sourcing and editing the clinical images and investigation results, drawing original diagrams and algorithms, and critically revising for important intellectual content: SF, BT, JV and NH. The following authors gave final approval of the manuscript: SF, BT, JV and NH.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.