Journal article
Biliary tract large cell neuroendocrine carcinoma: current evidence
Orphanet journal of rare diseases, Vol.14(1), 266
11/21/2019
DOI: 10.1186/s13023-019-1230-2
PMCID: PMC6869278
PMID: 31752927
Abstract
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.
Details
- Title: Subtitle
- Biliary tract large cell neuroendocrine carcinoma: current evidence
- Creators
- Riva Raiker - University of KentuckyAman Chauhan - University of KentuckyHassan Hasanein - University of KentuckyGrant Burkeen - University of KentuckyMillicent Horn - University of KentuckyJaneesh Veedu - University of KentuckyCory Vela - University of KentuckySusanne Arnold - University of KentuckyJill Kolesar - University of KentuckyLowell Anthony - University of KentuckyB. Mark Evers - University of KentuckyMichael Cavnar - University of Kentucky
- Resource Type
- Journal article
- Publication Details
- Orphanet journal of rare diseases, Vol.14(1), 266
- Publisher
- BioMed Central
- DOI
- 10.1186/s13023-019-1230-2
- PMID
- 31752927
- PMCID
- PMC6869278
- ISSN
- 1750-1172
- eISSN
- 1750-1172
- Language
- English
- Date published
- 11/21/2019
- Academic Unit
- Pharmacy; Pharmaceutical Sciences and Experimental Therapeutics
- Record Identifier
- 9984695803302771
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