Liver & intrahepatic bile ducts

Other malignancies

Neuroendocrine carcinoma



Topic Completed: 1 February 2012

Minor changes: 23 September 2020

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PubMed Search: Neuroendocrine carcinoma[TI] liver[TI]


Deepali Jain, M.D.
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Cite this page: Jain D. Neuroendocrine carcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livertumorNEcarcinoma.html. Accessed December 6th, 2021.
Definition / general
  • < 100 cases reported; mean age > 40 years, more common in women (Arch Pathol Lab Med 2003;127:1200)
  • Normal serum AFP but high serum NSE, chromogranin and synaptophysin levels
  • Not associated with chronic liver disease / cirrhosis
  • Presents with diarrhea and abdominal pain
  • Amphicrine carcinoma: glandular (signet ring morphology, mucicarmine, PASD+, villin+, CDX2+) and neuroendocrine differentiation in same cells (Ann Diagn Pathol 2011;15:355)
Case reports
Gross description
  • Typically solitary, may be cystic
Microscopic (histologic) description
  • Resembles poorly differentiated carcinomas with cellular pleomorphism, nuclear atypia, hyperchromasia and frequent mitotic figures
  • May have signet ring morphology with intracytoplasmic vacuoles negative for mucin but positive for neuroendocrine markers (Semin Liver Dis 2010;30:422)
Positive stains
Electron microscopy description
  • Dense core granules
Differential diagnosis
  • Carcinoid tumor: low (< 2/10 HPF) mitotic activity, minimal nuclear polymorphism
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