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Endoscopic Ultrasound-Guided Biliary Drainage: Endoscopic Ultrasound-Guided Hepaticogastrostomy in Malignant Biliary Obstruction
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Biliary tract large cell neuroendocrine carcinoma: current evidence
Riva Raiker1, Aman Chauhan2, Hassan Hasanein1
1Department of Internal Medicine, University of Kentucky, Lexington, KY, USA.
Orphanet Journal of Rare Diseases
|November 23, 2019
Summary
Large cell neuroendocrine carcinomas (LCNEC) of the biliary tract are rare and aggressive, often diagnosed late. Complete surgical excision and chemotherapy are mainstays, but prognosis remains poor with median survival of 11 months.
Area of Science:
- Hepatobiliary pathology
- Neuroendocrine tumors
- Oncology
Background:
- Primary neuroendocrine carcinomas of the gallbladder and biliary tract are rare.
- Pure large cell neuroendocrine carcinomas (LCNEC) are exceedingly rare and have a particularly poor prognosis.
Purpose of the Study:
- To review published data on biliary tract large cell neuroendocrine carcinomas.
- To summarize the current understanding of diagnosis, treatment, and prognosis for this rare cancer.
Main Methods:
- PubMed search for published data on biliary tract LCNEC.
- Analysis of 12 reported cases of pure biliary tract LCNEC.
Main Results:
- Biliary tract LCNEC presents with non-specific symptoms, leading to late diagnosis post-resection.
- These cancers are aggressive with high recurrence and metastasis rates, often to lymph nodes and liver.
- Complete surgical excision with chemotherapy is the primary treatment for resectable cases; chemotherapy is used for unresectable disease.
Conclusions:
- The prognosis for hepatobiliary LCNEC is poor, with a median survival of 11 months.
- Further research into high-grade neuroendocrine carcinomas is needed to improve understanding and therapeutics.
