Kyong Joo Lee1, Jae Hee Cho2, Sang Hyub Lee3, Kwang Hyuk Lee4, Byung Kyu Park5, Jun Kyu Lee6, Sang Myung Woo7, Ji Kon Ryu3, Jong Kyun Lee4, Yeon Suk Kim2, Jae Woo Kim1, Woo Jin Lee7. 1. a Department of Internal Medicine , Yonsei University Wonju College of Medicine , Wonju , South Korea. 2. b Department of Internal Medicine , Gachon university, Gil Medical Center , Incheon , South Korea. 3. c Department of Internal Medicine and Liver Research Institute , Seoul National University College of Medicine , Seoul , South Korea. 4. d Department of Internal Medicine , Samsung Medical Center, Sungkyunkwan University School of Medicine , Seoul , South Korea. 5. e National Health Insurance Corporation Ilsan Hospital , Goyang , South Korea. 6. f Department of Internal Medicine , Dongguk University Ilsan Hospital, Dongguk University , Goyang , South Korea. 7. g Center for Liver Cancer, National Cancer Center , Goyang , South Korea.
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.
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.
Entities:
Keywords:
World Health Organization classification; biliary tract; neuroendocrine carcinoma; neuroendocrine neoplasm; prognosis
Authors: Shekhar Gogna; David Samson; Mahir Gachabayov; Aram Rojas; Daniel Moritz Felsenreich; Donna Koo; Katie Gu; Luis Quintero; Kendall Ryan Miller; Asad Azim; Xiang Da Eric Dong Journal: Langenbecks Arch Surg Date: 2021-07-08 Impact factor: 2.895