Biliary tract large cell neuroendocrine carcinoma: Current evidence

Riva Raiker, Aman Chauhan, Hassan Hasanein, Grant Burkeen, Millicent Horn, Janeesh Veedu, Cory Vela, Susanne Arnold, Jill Kolesar, Lowell Anthony, B. Mark Evers, Michael Cavnar

Research output: Contribution to journalReview articlepeer-review

9 Scopus citations


Background: Primary neuroendocrine carcinomas of the gallbladder and biliary tract are rare, with pure large cell neuroendocrine carcinomas (LCNEC) being exceedingly rare and with a particularly poor prognosis. Methods: We performed a review of published data on biliary tract large cell neuroendocrine carcinomas in PubMed. Results: Preliminary search revealed over 2000 results but we found only 12 cases of pure large cell neuroendocrine carcinomas of biliary tract noted in literature to date. Because it commonly presents with non-specific symptoms of abdominal pain and jaundice, diagnosis is made after resection with histo-pathological and immunohistochemical analysis. These cancers are particularly aggressive with high recurrence rates, most often presenting with metastasis to regional lymph nodes and/or the liver resulting in a poor prognosis. Overall, complete surgical excision with systemic chemotherapy is the treatment mainstay. If the cancer is unresectable due to multiple metastases, medical management with systemic chemotherapy is the primary treatment modality. Conclusion: The prognosis of hepatobiliary LCNEC remains poor with median survival of only 11 months from initial diagnosis. Studies focusing on high grade neuroendocrine carcinoma are needed to enhance our understanding of biology and therapeutics in this rare but aggressive cancer.

Original languageEnglish
Article number266
JournalOrphanet Journal of Rare Diseases
Issue number1
StatePublished - Nov 21 2019

Bibliographical note

Publisher Copyright:
© 2019 The Author(s).


  • Biliary tract
  • Large cell neuroendocrine carcinomas
  • Neuroendocrine

ASJC Scopus subject areas

  • Genetics(clinical)
  • Pharmacology (medical)


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