Surgical Management of Extrahepatic Biliary Neuroendocrine Tumors: A Case Report
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Abstract
Extrahepatic biliary neuroendocrine tumors (EBNETs) are a rare group of neoplasms with varying characteristics, with 223 cases documented since their discovery. In this case report, an EBNET is described as a result of vague abdominal pain and significantly elevated liver function tests in a 41-year-old woman who presented with vague abdominal pain and elevated liver function tests. Despite the challenges faced in preoperative diagnosis, including the need to differentiate from cholangiocarcinoma, surgical intervention was successful. Due to the tumor’s complex nature, meticulous dissection and reconstruction were required, leading to crucial insights into surgical management. In addition to highlighting the rarity of EBNETs, this case emphasizes the importance of early detection and customized surgical approaches.
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Dasari A, Shen C, Halperin D, Zhao B, Zhou S, Xu Y, et al. Trends in the Incidence, Prevalence, and Survival Outcomes in Patients With Neuroendocrine Tumors in the United States. JAMA Oncol. 2017;3(10):1335-1342. Available from: https://doi.org/10.1001/jamaoncol.2017.0589
Dominguez DA, Eade AV, Aversa JG, Hagerty BL, Blakely AM, Davis JL, et al. Extrahepatic biliary neuroendocrine tumors: A national cancer database analysis. Heliyon. 2024;10(15):e34714. Available from: https://doi.org/10.1016/j.heliyon.2024.e34714
Taj R, Perez S, Jih LJ, Huynh C, Berumen J, Bouvet M. Primary extrahepatic biliary neuroendocrine tumor: a case report. J Surg Case Rep. 2023 May 1;2023(5):rjad277. Available from: https://doi.org/10.1093/jscr/rjad277
Gusani NJ, Marsh JW, Nalesnik MA, Tublin ME, Gamblin TC. Carcinoid of the extra-hepatic bile duct: a case report with long-term follow-up and review of literature. Am Surg. 2008;74(1):87-90. Available from: https://pubmed.ncbi.nlm.nih.gov/18274439/
El Rassi ZS, Mohsine RM, Berger F, Thierry P, Partensky CCM. Endocrine tumors of the extrahepatic bile ducts. Pathological and clinical aspects, surgical management and outcome. Hepatogastroenterology. 2004 Sep 1;51(59):1295-300. Available from: https://pubmed.ncbi.nlm.nih.gov/15362737/
Satturwar SP, Pantanowitz JL, Manko CD, Seigh L, Monaco SE, Pantanowitz L. Ki-67 proliferation index in neuroendocrine tumors: Can augmented reality microscopy with image analysis improve scoring? Cancer Cytopathol. 2020;128(8):535-44. Available from: https://doi.org/10.1002/cncy.22272
Brown WM, Henderson JM, Kennedy JC. Carcinoid tumor of the bile duct. A case report and literature review. Am Surg. 1990 Jun;56(6):343-6. Available from: https://pubmed.ncbi.nlm.nih.gov/2190510/
Govind D, Jen KY, Matsukuma K, Gao G, Olson KA, Gui D, et al. Improving the accuracy of gastrointestinal neuroendocrine tumor grading with deep learning. Sci Rep. 2020;10(1):11064. Available from: https://doi.org/10.1038/s41598-020-67880-z
Michalopoulos N, Papavramidis TS, Karayannopoulou G, Pliakos I, Papavramidis ST, Kanellos I. Neuroendocrine Tumors of Extrahepatic Biliary Tract. Pathol Oncol Res. 2014;20(4):765-75. Available from: https://doi.org/10.1007/s12253-014-9808-4
Noronha YS, Raza AS. Well-differentiated neuroendocrine (carcinoid) tumors of the extrahepatic biliary ducts. Arch Pathol Lab Med. 2010;134(7):1075-9. Available from: https://doi.org/10.5858/2008-0764-rs.1
Abe T, Nirei A, Suzuki N, Todate Y, Azami A, Waragai M, et al. Neuroendocrine tumor of the extrahepatic bile duct: A case report. Int J Surg Case Rep. 2017 Sep 7;40:6-9. Available from: https://doi.org/10.1016/j.ijscr.2017.09.001
Nwankwo EC, Khneizer G, Sayuk G, Presti M, Elwing JE. S1770 Neuroendocrine Tumor in the Biliary Tract Presenting With Cholangitis. Am J Gastroenterol. 2022 Oct;117(10S):e1245. Available from: http://dx.doi.org/10.14309/01.ajg.0000863720.99786.cf
Dominguez D, Hagerty B, Aversa J, Melstrom L, Hernandez J. Extrahepatic bile duct neuroendocrine tumors: casting a wide net with a large national database. HPB. 2023;25:S52. Available from: https://doi.org/10.1016/j.hpb.2023.05.101
Chopde A, Gupta V, Jadhav A, Kumar R, Ramadwar M, Patkar S, et al. Neuroendocrine Tumours of Extrahepatic Biliary Tract: Report of Four Cases with Literature Review. Indian J Surg Oncol. 2024 May 1;15(2):212-7. Available from: https://doi.org/10.1007/s13193-022-01621-8
Liu Z, Zhang DY, Lu Z, Zhang P, Sun WL, Ma X, et al. Neuroendocrine tumor of the common bile duct: a case report and review of the literature. OncoTargets Ther. 2018;11:2295-301. Available from: https://doi.org/10.2147/ott.s162934
Todoroki T, Sano T, Yamada S, Hirahara N, Toda N, Tsukada K, et al. Clear cell carcinoid tumor of the distal common bile duct. World J Surg Oncol. 2007;5:6. Available from: https://doi.org/10.1186/1477-7819-5-6
Tzimas GN, Vali K, Deschênes M, Marcus VA, Barkun JS, Tchervenkov JI, et al. Liver transplantation for metastases from a bile duct carcinoid. HPB (Oxford). 2006;8(1):67-8. Available from: https://doi.org/10.1080/13651820600573170
Walter T, Brixi-Benmansour H, Lombard-Bohas C, Cadiot G. New treatment strategies in advanced neuroendocrine tumors. Dig Liver Dis. 2012 Feb 1;44(2):95-105. Available from: https://doi.org/10.1016/j.dld.2011.08.022