Clinical Journal of Gastroenterology

, Volume 6, Issue 2, pp 177–187

Carcinoid tumor of the extrahepatic bile duct: report of a case

  • Takeo Yasuda
  • Gen Imai
  • Masako Takemoto
  • Mitsuo Yamasaki
  • Hajime Ishikawa
  • Masayuki Kitano
  • Takuya Nakai
  • Yoshifumi Takeyama
Case Report


We report a rare case of carcinoid tumor of the extrahepatic bile duct. A 69-year-old woman with a history of hyperthyroidism was diagnosed to have a tumor of the extrahepatic bile duct. Laparotomy, for presumed cholangiocarcinoma, revealed a 2.5-cm-long, firm mass of the hilar-upper bile duct. The extrahepatic bile duct resection and lymphadenectomy was performed. Her postoperative course was uneventful and has been asymptomatic without recurrent tumor during 2 years of follow-up. Primary carcinoid tumors of the extrahepatic bile duct are very rare. Herein we report this rare case with a review of the literature


Carcinoid tumor Bile duct Neuroendocrine tumor 


  1. 1.
    Oberndorfer S. Karzenoide Tumoren des Dunndarms. Frankf Zschr Path. 1907;1:426–30.Google Scholar
  2. 2.
    Gosset A, Masson P. Tumeurs endocrines de l’appendice. Presse Med. 1914;25:237–9.Google Scholar
  3. 3.
    Klimstra DS, Modlin IR, Coppola D, Lloyd RV, Suster S. The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas. 2010;39:707–12.PubMedCrossRefGoogle Scholar
  4. 4.
    Todoroki T, Kawamoto T, Koike N, Fukao K, Shoda J, Takahashi H. Treatment strategy for patients with middle and lower third bile duct cancer. Br J Surg. 2001;88:364–70.PubMedCrossRefGoogle Scholar
  5. 5.
    Todoroki T, Kawamoto T, Koike N, Takahashi H, Yoshida S, Kashiwagi H, et al. Radical resection of hilar bile duct carcinoma and predictors of survival. Br J Surg. 2000;87:306–13.PubMedCrossRefGoogle Scholar
  6. 6.
    Modlin IM, Shapiro MD, Kidd M. An analysis of rare carcinoid tumors: clarifying these clinical conundrums. World J Surg. 2005;29:92–101.PubMedCrossRefGoogle Scholar
  7. 7.
    Davies AJ. Carcinoid tumors (argentaffinomata). Ann R Coll Surg Engl. 1959;25:277–80.PubMedGoogle Scholar
  8. 8.
    Pilz E. Uber ein karzinoid des ductus choledochus. Zentralbl Chir. 1961;86:1588–90.PubMedGoogle Scholar
  9. 9.
    Little JM, Gibson AA, Kay AW. Primary common bile-duct carcinoid. Br J Surg. 1968;55:147–9.PubMedCrossRefGoogle Scholar
  10. 10.
    Godwin JD. Carcinoid tumours. An analysis of 2,837 cases. Cancer. 1975;36:560–9.PubMedCrossRefGoogle Scholar
  11. 11.
    Bergdahl L. Carcinoid tumours of the biliary tract. Aust N Z J Surg. 1976;46:136–8.PubMedCrossRefGoogle Scholar
  12. 12.
    Judge DM, Dickman PS, Trapukdi BS. Nonfunctioning argyrophilic tumour (APUDoma) of the hepatic duct: simplified methods of detecting biogenic amines in tissue. Am J Clin Pathol. 1976;66:40–5.PubMedGoogle Scholar
  13. 13.
    Gerlock AJ Jr, Muhletaler CA. Primary common bile duct carcinoid. Gastrointest Radiol. 1979;4:263–4.PubMedCrossRefGoogle Scholar
  14. 14.
    Vitaux J, Salmon RJ, Languille O, Buffet C, Martin E, Chaput JC. Carcinoid tumor of the common bile duct. Am J Gastroenterol. 1981;76:360–2.PubMedGoogle Scholar
  15. 15.
    Abe V, Itoh H, Yoshida M. Carcinoid tumor of the common bile duct: report of a case. Stomach Intest. 1983;18:543–8.Google Scholar
  16. 16.
    Goodman ZD, Albores-Saavedra J, Lundblad DM. Somatostatinoma of the cystic duct. Cancer. 1984;53:498–502.PubMedCrossRefGoogle Scholar
  17. 17.
    Alexander IA, Thomson KR, Kune GA. Primary common bile duct carcinoid: demonstration by computerised tomography, ultrasonography and angiography. Australas Radiol. 1986;30:34–7.PubMedCrossRefGoogle Scholar
  18. 18.
    Jutte DL, Bell RH Jr, Penn I, Powers J, Kolinjivadi J. Carcinoid tumor of the biliary system. Case report and literature review. Dig Dis Sci. 1986;32:763–9.Google Scholar
  19. 19.
    Nicolescu PG, Popescu A. Carcinoid tumor of the cystic duct. Morphol Embryol. 1986;32:275–7.Google Scholar
  20. 20.
    Bickerstaff DR, Ross WB. Carcinoid of the biliary tree: a case report and review of the literature. J R Coll Surg Edinb. 1987;32:48–51.PubMedGoogle Scholar
  21. 21.
    Fujita N, Mochizuki F, Lee S, Satoh K, Kobayashi G, Yano A, et al. Carcinoid tumor of the bile duct: case report. Gastrointest Radiol. 1989;14:151–4.PubMedCrossRefGoogle Scholar
  22. 22.
    Chittal SM, Ra PM. Carcinoid of the cystic duct. Histopathology. 1989;15:643–6.PubMedCrossRefGoogle Scholar
  23. 23.
    van der Wal AC, Van Leeuwen DJ, Walford N. Small cell neuroendocrine (oat cell) tumor of the common bile duct. Histopathology. 1989;16:398–400.CrossRefGoogle Scholar
  24. 24.
    Bumin C, Ormeci N, Dolapci M, Gungor S. Carcinoid tumor of the biliary duct. Int Surg. 1990;75:262–4.PubMedGoogle Scholar
  25. 25.
    Brown WM, Henderson JM, Kennedy JC. Carcinoid tumor of the bile duct. A case report and literature review. Am Surg. 1990;56:343–6.PubMedGoogle Scholar
  26. 26.
    Fellows IW, Leach IH, Smith PG, Toghill PJ, Doran J. Carcinoid tumor of the common bile duct-a novel complication of von Hippel–Lindau syndrome. Gut. 1990;31:728–9.PubMedCrossRefGoogle Scholar
  27. 27.
    Angeles-Angeles A, Quintanilla-Martìnez L, Larriva-Sahd J. Primary carcinoid the common bile duct. Immunohistochemical characterization of a case and review of the literature. Am J Clin Pathol. 1991;96:341–4.PubMedGoogle Scholar
  28. 28.
    Besznyak I, Tasnadi G, Hirsch T, Koves I, Schaff Z, Lapis K. Hepato-lobectomy for carcinoid tumor of the hepatic duct. Orv Hetil. 1991;132:591–4.PubMedGoogle Scholar
  29. 29.
    Barron-Rodriguez LP, Manivel JC, Mendez-Sanchez N, Jessurun J. Carcinoid tumor of the common bile duct: evidence for its origin in metaplastic endocrine cells. Am J Gastroenterol. 1991;86:1073–6.PubMedGoogle Scholar
  30. 30.
    Newman K, Vates T, Duffy L, Anderson K. Pancreatoduodenectomy with preservation of the stomach and pylorus: a safe and effective alternative in children. J Pediatr Surg. 1992;27:1334–5.PubMedCrossRefGoogle Scholar
  31. 31.
    Dixon J, Lisehora G, Lee MY. Carcinoid tumor of the common bile duct. Contemp Surg. 1992;41:37–40.Google Scholar
  32. 32.
    Rugge M, Sonego F, Militello C, Guido M, Ninfo V. Primary carcinoid tumor of the cystic and common bile ducts. Am J Surg Pathol. 1992;16:802–7.PubMedCrossRefGoogle Scholar
  33. 33.
    Ueyama T, Ding J, Hashimoto H, Tsuneyoshi M, Enjoji M. Carcinoid tumor arising in the wall of a congenital bile duct cyst. Arch Pathol Lab Med. 1992;116:291–3.PubMedGoogle Scholar
  34. 34.
    Gembala RB, Arsuaga JE, Friedman AC, Radecki PD, Ball DS, Hartman GG, Rabin L, Caroline DF. Carcinoid of the intrahepatic ducts. Abdom Imaging. 1993;18(3):242–4.PubMedCrossRefGoogle Scholar
  35. 35.
    Sankary HN, Foster P, Frye E, Williams JW. Carcinoid tumours of the extrahepatic bile duct: an unusual cause of bile duct obstruction. Liver Transpl Surg. 1995;1:122–3.PubMedCrossRefGoogle Scholar
  36. 36.
    Mandujano-Vera G, Angeles-Angeles A, De la Cruz-Hernandez J, Sansores-Perez M, LarrivaSahd J. Gastrinoma of the common bile duct: immunohistochemical and ultrastructural study of a case. J Clin Gastroenterol. 1995;20:321–4.PubMedCrossRefGoogle Scholar
  37. 37.
    Belli G, Rotondano G, D’Agostino A, Marano I. Primary extrahepatic bile duct carcinoids. HPB Surg. 1996;9:101–5.Google Scholar
  38. 38.
    Kopelman D, Schein M, Kerner H, Bahuss H, Hashmonai M. Carcinoid tumor of the common bile duct. HPB Surg. 1996;10:41–3.PubMedCrossRefGoogle Scholar
  39. 39.
    Hao L, Friedman AL, Navarro VJ, West B, Robert ME. Carcinoid tumor of the common bile duct producing gastrin and serotonin. J Clin Gastroenterol. 1996;23:63–5.PubMedCrossRefGoogle Scholar
  40. 40.
    Nahas SC, Lourencao JL, Gazoni E, Mott Cde B, Borba MR, Pinto Filho JO, et al. Carcinoid tumors of the common bile duct: report of a case. Rev Hosp Clin Fac Med Sao Paulo. 1998;53:26–8.Google Scholar
  41. 41.
    Shah IA, Schlageter MO, Wong SX, Gani OS. Carcinoid tumor of the cystic duct. Dig Surg. 1998;15:372–3.PubMedCrossRefGoogle Scholar
  42. 42.
    Bembenek A, Lotterer E, Machens A, Cario H, Krause U, Holzhausen HJ, et al. Neuroendocrine tumor of the common hepatic duct: a rare case of extrahepatic jaundice in adolescence. Surgery. 1998;123:712–5.PubMedCrossRefGoogle Scholar
  43. 43.
    Ross AC, Hurley JB, Hay WB, Rusnak CH, Petrunia DM. Carcinoids of the common bile duct: a case report and literature review. Can J Surg. 1999;42:59–63.PubMedGoogle Scholar
  44. 44.
    Perakath B, Chandran BS, Srivastava VM, Nair A, Sitaram V. Carcinoid tumor of common hepatic duct. Indian J Gastroenterol. 1999;18:127.PubMedGoogle Scholar
  45. 45.
    Hermina M, Starling J, Warner TF. Carcinoid tumor of the cystic duct. Pathol Res Pract. 1999;195:707–10.PubMedCrossRefGoogle Scholar
  46. 46.
    Chamberlain RS, Blumgart LH. Carcinoid tumors of the extrahepatic bile duct. A rare cause malignant biliary obstruction. Cancer. 1999;86:1956–65.CrossRefGoogle Scholar
  47. 47.
    Chan C, Medina-Franco H, Bell W, Lazenby A, Vickers S. Carcinoid tumor of the hepatic duct presenting as a Klatskin tumor in an adolescent and review of world literature. Hepatogastroenterology. 2000;47:519–21.PubMedGoogle Scholar
  48. 48.
    Juturi JV, Maghfoor I, Doll DC, Evans ML. A case of biliary carcinoid presenting with pancreatitis and obstructive jaundice. Am J Gastroenterol. 2000;95:2973–4.PubMedCrossRefGoogle Scholar
  49. 49.
    Maitra A, Krueger JE, Tascilar M, Offerhaus GJ, Angeles-Angeles A, Klimstra DS, et al. Carcinoid tumors of the extrahepatic bile ducts: a study of seven cases. Am J Surg Pathol. 2000;24:1501–10.PubMedCrossRefGoogle Scholar
  50. 50.
    Turrión VS, Salas C, Alvira LG, Jimenez M, Lucena JL, Ardaiz J. Carcinoid tumour of the common bile duct: an exceptional indication for liver transplantation. Transplant Proc. 2002;34:264–5.PubMedCrossRefGoogle Scholar
  51. 51.
    Volpe CM, Pryor JP, Caty M, Doerr RJ. An adolescent with bile duct carcinoid tumor. Med Pediatr Oncol. 2003;40:137–8.PubMedCrossRefGoogle Scholar
  52. 52.
    Pawlik TM, Shah S, Eckhauser FE. Carcinoid tumor of the biliary tract: treating a rare cause of duct obstruction. Am Surg. 2003;69:98–101.PubMedGoogle Scholar
  53. 53.
    Podnos YD, Jimenez JC, Zainabadi K, Ji P, Cooke J, Busuttil RW, et al. Carcinoid tumors of the common bile duct: report of two cases. Surg Today. 2003;33:553–5.PubMedGoogle Scholar
  54. 54.
    El Rassi ZS, Mohsine RM, Berger F, Thierry P, Partensky CC. Endocrine tumors of the extrahepatic bile ducts. Pathological and clinical aspects, surgical management and outcome. Hepatogastroenterology. 2004;51:1295–300.PubMedGoogle Scholar
  55. 55.
    Menezes AA, Diver AJ, McCance D, Diamond T. Carcinoid tumor of the extrahepatic bile duct—report of a case and literature review. Ulster Med J. 2004;73:59–62.PubMedGoogle Scholar
  56. 56.
    Ligato S, Furmaga W, Cartun RW, Hull D, Tsongalis GJ. Primary carcinoid tumor of the common hepatic duct: a rare case with immunohistochemical and molecular findings. Oncol Rep. 2005;13:543–6.PubMedGoogle Scholar
  57. 57.
    Pithawala M, Mittal G, Prabhu RY, Kantharia CV, Joshi A, Supe A. Carcinoid tumor of bile duct. Indian J Gastroenterol. 2005;24:262–3.PubMedGoogle Scholar
  58. 58.
    Hubert C, Sempoux C, Berquin A, Deprez P, Jamar F, Gigot JF. Bile duct carcinoid tumors: an uncommon disease but with a good prognosis? Hepatogastroenterology. 2005;52:1042–7.PubMedGoogle Scholar
  59. 59.
    Nesi G, Lombardi A, Batignani G, Ficari F, Rubio CA, Tonelli F. Well-differentiated endocrine tumor of the distal common bile duct: a case study and literature review. Virchows Arch. 2006;449:104–11.PubMedCrossRefGoogle Scholar
  60. 60.
    Caglikulekci M, Dirlik M, Aydin O, Colak T, Dag A, Canbaz H, et al. Carcinoid tumour of the common bile duct: report of a case and a review of the literature. Acta Chir Belg. 2006;106:112–5.PubMedGoogle Scholar
  61. 61.
    Tzimas GN, Vali K, Deschenes M, Marcus VA, Barkun JS, Tchervenkov JI, et al. Liver transplantation for metastases from a bile duct carcinoid. HPB. 2006;8:67–8.PubMedCrossRefGoogle Scholar
  62. 62.
    Kim DH, Song MH, Kim DH. Malignant carcinoid tumor of the common bile duct: report of a case. Surg Today. 2006;36(5):485–9.PubMedCrossRefGoogle Scholar
  63. 63.
    Ferrone CR, Tang LH, D’Angelica M, De Matteo RP, Blumgart LH, Klimstra DS, et al. Extrahepatic bile duct carcinoid tumors: malignant biliary obstruction with a good prognosis. J Am Coll Surg. 2007;205:357–61.PubMedCrossRefGoogle Scholar
  64. 64.
    Todoroki T, Sano T, Yamada S, Hirahara N, Toda N, Tsukada K, Motojima R, Motojima T. Clear cell carcinoid tumor of the distal common bile duct. World J Surg Oncol. 2007;17(5):6.CrossRefGoogle Scholar
  65. 65.
    Sethi H, Madanur M, Srinivasan P, Portmann B, Heaton N, Rela M. Non-functioning well-differentiated neuroendocrine tumor of the extrahepatic bile duct: an unusual suspect? Hepatobiliary Pancreat Dis Int. 2007;6:549–52.PubMedGoogle Scholar
  66. 66.
    Costantini M, Montalti R, Rossi G, Losi L, Masetti M, Di Benedetto F, et al. Adenocarcinoid tumor of the extrahepatic biliary tract. Int J Surg Pathol. 2008;16:455–7.PubMedCrossRefGoogle Scholar
  67. 67.
    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;74:87–90.PubMedGoogle Scholar
  68. 68.
    Nafidi O, Nguyen BN, Roy A. Carcinoid tumor of the common bile duct: a rare complication of von Hippel–Lindau syndrome. World J Gastroenterol. 2008;14:1299–301.PubMedCrossRefGoogle Scholar
  69. 69.
    Schmitt TM, Bonatti H, Hagspiel KD, Iezzoni J, Northup P, Pruett TL. Carcinoid of the bile duct bifurcation. J Am Coll Surg. 2008;206:399.PubMedCrossRefGoogle Scholar
  70. 70.
    Felekouras E, Petrou A, Bramis K, Prassas E, Papaconstantinou I, Dimitriou N, et al. Malignant carcinoid tumor of the cystic duct: a rare cause of bile duct obstruction. Hepatobiliary Pancreat Dis Int. 2009;8:640–6.PubMedGoogle Scholar
  71. 71.
    Squillaci S, Marchione R, Piccolomini M, Colombo F, Bucci F, Bruno M, et al. Well-differentiated neuroendocrine carcinoma (malignant carcinoid) of the extrahepatic biliary tract: report of two cases and literature review. APMIS. 2010;118:543–56.PubMedGoogle Scholar
  72. 72.
    Zhan J, Bao G, Hu X, Gao W, Ruo X, Gong J, et al. Carcinoid tumor of the common bile duct in children: a case report. J Pediatr Surg. 2010;45:2061–3.PubMedCrossRefGoogle Scholar
  73. 73.
    Johnson G, Hatfield A, Webster G, Groves C, Rodriguez-Justo M. A diagnosis of an intraluminal carcinoid tumor of the bile duct by using cholangioscopy. Gastrointest Endosc. 2010;71:622–3.PubMedCrossRefGoogle Scholar
  74. 74.
    Lee JH, Lee KG, Oh YH, Paik SS, Park HK, Lee KS. Carcinoid tumors of the extrahepatic biliary tract: report of four cases. Surg Today. 2011;41:430–5.PubMedCrossRefGoogle Scholar
  75. 75.
    Bhalla P, Powle V, Shah RC, Jagannath P. Neuroendocrine tumor of common hepatic duct. Indian J Gastroenterol. 2012;31:144–6.PubMedCrossRefGoogle Scholar
  76. 76.
    Takahashi K, Sasaki R, Oshiro Y, Fukunaga K, Oda T, Ohkohchi N. Well-differentiated endocrine carcinoma originating from the bile duct in association with a congenital choledochal cyst. Int Surg. 2012;97:315–20.PubMedCrossRefGoogle Scholar
  77. 77.
    Jethava A, Muralidharan V, Mesologites T, Stoica-Mustafa E, Dasanu CA. An unusual presentation of a carcinoid tumor of the common bile duct. JOP. 2013;14:85–7.PubMedGoogle Scholar
  78. 78.
    Eloubeidi MA, Chen VK, Jhala NC, Eltoum IE, Jhala D, Chhieng DC, et al. Endoscopic ultrasound-guided fine needle aspiration biopsy of suspected cholangiocarcinoma. Clin Gastroenterol Hepatol. 2004;2:209–13.PubMedCrossRefGoogle Scholar
  79. 79.
    Byrne MF, Gerke H, Mitchell RM, Stiffler HL, McGrath K, Branch MS, et al. Yield of endoscopic ultrasound-guided fine-needle aspiration of bile duct lesions. Endoscopy. 2004;36:715–9.PubMedCrossRefGoogle Scholar
  80. 80.
    Meara RS, Jhala D, Eloubeidi MA, Eltoum I, Chhieng DC, Crowe DR, et al. Endoscopic ultrasound-guided FNA biopsy of bile duct and gallbladder: analysis of 53 cases. Cytopathology. 2006;17:42–9.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Japan 2013

Authors and Affiliations

  • Takeo Yasuda
    • 1
  • Gen Imai
    • 2
  • Masako Takemoto
    • 1
  • Mitsuo Yamasaki
    • 1
  • Hajime Ishikawa
    • 1
  • Masayuki Kitano
    • 2
  • Takuya Nakai
    • 1
  • Yoshifumi Takeyama
    • 1
  1. 1.Department of SurgeryKinki University Faculty of MedicineOsaka-sayamaJapan
  2. 2.Division of Hepatology and Pancreatology, Department of Internal MedicineKinki University Faculty of MedicineOsaka-sayamaJapan

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