Clinicopathological characteristics of biliary neuroendocrine neoplasms: a multicenter study |
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| Authors: | Kyong Joo Lee Jae Hee Cho Sang Hyub Lee Kwang Hyuk Lee Byung Kyu Park Jun Kyu Lee |
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| Institution: | 1. Department of Internal Medicine, Yonsei University Wonju College of Medicine, Wonju, South Korea;2. Department of Internal Medicine, Gachon university, Gil Medical Center, Incheon, South Korea;3. Department of Internal Medicine and Liver Research Institute, Seoul National University College of Medicine, Seoul, South Korea;4. Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea;5. National Health Insurance Corporation Ilsan Hospital, Goyang, South Korea;6. Department of Internal Medicine, Dongguk University Ilsan Hospital, Dongguk University, Goyang, South Korea |
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| Abstract: | Objectives: This study assessed the clinicopathological features, therapeutic approaches, and prognosis of patients with biliary neuroendocrine neoplasm (NENs).Materials and methods: Multicenter retrospective study of patients with biliary tract NENs in the gallbladder, the extrahepatic bile duct, or the ampulla of Vater between 2005 and 2014.Results: Total of 43 patients were included in the study. The median age was 62 years (range: 29–84 years) and 58.1% of the patients were male. The tumors occurred in the gallbladder (n?=?11), the extrahepatic bile duct (n?=?5) or the ampulla of Vater (n?=?27). The liver was the most common metastatic site. Based on the 2010 World Health Organization classification, more patients with gallbladder NENs (11/11 (100%)) had neuroendocrine carcinoma G3 than those with NENs in the ampulla of Vater (10/27 (37.1%)). The median progression free survival time (39.3 vs 5.1 months, p?=?0.001) and median overall survival time (46.9 vs 7.9 months, p?<?0.001) were significantly longer in patients with ampulla of Vater NENs than gallbladder NENs. A 2010 World Health Organization classification of neuroendocrine carcinoma G3 was independently related to poor overall survival (hazard ratio (HR), 27.1; 95% confidence intervals (CI), 2.81-260.68; p?=?0.004).Conclusion: The 2010 World Health Organization classification of neuroendocrine carcinoma G3 was the only factor related to poor prognosis in patients with biliary neuroendocrine neoplasms. |
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| Keywords: | World Health Organization classification biliary tract neuroendocrine neoplasm neuroendocrine carcinoma prognosis |
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