Advertisement

Miscellaneous Nonpancreatic Nonendocrine Tumors

  • Heather A. Lillemoe
  • John D. Abad
  • Keith D. Lillemoe
Reference work entry

Abstract

Nonendocrine, nonpancreatic periampullary tumors are generally classified as arising from the ampulla of Vater, distal common bile duct, or duodenum. The most common clinical finding on presentation is obstructive jaundice. These lesions may occur spontaneously or as part of a hereditary syndrome (familial adenomatous polyposis, Gardner’s syndrome, and inflammatory bowel disease). The most effective diagnostic strategies for determining extent of disease and resectability of periampullary tumors include dual-phase computed tomography and endoscopic ultrasound. Small, benign periampullary lesions may be amenable to endoscopic resection. For benign lesions <3 cm that are unable to be completely removed endoscopically, transduodenal local resection should be considered. Appropriate surgical candidates with larger lesions >3 cm or suspicion of invasive carcinoma should undergo a pancreaticoduodenectomy. Five-year survival for duodenal, ampullary, and distal common bile duct carcinomas are 51–59, 37–39, and 23–27%, respectively. For each of these tumors, both lymph node status and negative margins are significant predictors of outcome. At this point, neoadjuvant and adjuvant therapies have not clearly demonstrated a survival benefit for nonpancreatic periampullary cancers. The future success in treating these cancers likely rests in the development of novel biological and targeted therapies in the setting of well-designed multi-institutional clinical trials. This chapter will focus on benign and malignant nonpancreatic and nonneuroendocrine periampullary tumors and will include the pathology, clinical presentation, diagnostic workup, and management strategies to approach these neoplasms.

Keywords

Cholangiocarcinoma Bile duct cancer Ampullary adenoma Ampullary cancer Duodenal neoplams 

References

  1. 1.
    Riall TS, et al. Pancreaticoduodenectomy with or without distal gastrectomy and extended retroperitoneal lymphadenectomy for periampullary adenocarcinoma – part 3: update on 5-year survival. J Gastrointest Surg. 2005;9(9):1191–204.CrossRefGoogle Scholar
  2. 2.
    Riall TS, et al. Resected periampullary adenocarcinoma: 5-year survivors and their 6- to 10-year follow-up. Surgery. 2006;140(5):764–72.CrossRefGoogle Scholar
  3. 3.
    Chini P, Draganov PV. Diagnosis and management of ampullary adenoma: the expanding role of endoscopy. World J Gastrointest Endosc. 2011;3(12):241–7.CrossRefGoogle Scholar
  4. 4.
    Panzeri F, Crippa S, Castelli P, et al. Management of ampullary neoplasms: a tailored approach between endoscopy and surgery. World J Gastroenterol. 2015;21(26):7970–87.CrossRefGoogle Scholar
  5. 5.
    Blackman E, Nash SV. Diagnosis of duodenal and ampullary epithelial neoplasms by endoscopic biopsy: a clinicopathologic and immunohistochemical study. Hum Pathol. 1985;16(9):901–10.CrossRefGoogle Scholar
  6. 6.
    Yamaguchi K, Enjoji M. Carcinoma of the ampulla of Vater. A clinicopathologic study and pathologic staging of 109 cases of carcinoma and 5 cases of adenoma. Cancer. 1987;59(3):506–15.CrossRefGoogle Scholar
  7. 7.
    Galandiuk S, et al. Villous tumors of the duodenum. Ann Surg. 1988;207(3):234–9.CrossRefGoogle Scholar
  8. 8.
    Mino M, Lauwers GY. Pathology of periampullary tumors. In: Von Hoff DD, Evans DB, Hruban RH, editors. Pancreatic cancer. Boston: Jones and Bartlett; 2005. p. 686–702.Google Scholar
  9. 9.
    Carter JT, et al. Tumors of the ampulla of Vater: histopathologic classification and predictors of survival. J Am Coll Surg. 2008;207(2):210–8.CrossRefGoogle Scholar
  10. 10.
    Komorowski RA, et al. Assessment of ampulla of Vater pathology. An endoscopic approach. Am J Surg Pathol. 1991;15(12):1188–96.CrossRefGoogle Scholar
  11. 11.
    Devaney K, Goodman ZD, Ishak KG. Hepatobiliary cystadenoma and cystadenocarcinoma. A light microscopic and immunohistochemical study of 70 patients. Am J Surg Pathol. 1994;18(11):1078–91.CrossRefGoogle Scholar
  12. 12.
    Barton JG, Barrett DA, Maricevich MA, et al. Intraductal papillary mucinous neoplasm of the biliary tract: a real disease? HPB (Oxford). 2009;11(8):684–91.CrossRefGoogle Scholar
  13. 13.
    Everhart JE, Ruhl CE. Burden of digestive diseases in the United States part III: liver, biliary tract, and pancreas. Gastroenterology. 2009;136(4):1134–44.CrossRefGoogle Scholar
  14. 14.
    Nakeeb A, et al. Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors. Ann Surg. 1996;224(4):463–73.CrossRefGoogle Scholar
  15. 15.
    Cardinale V, Semeraro R, Torrice A, et al. Intra-hepatic and extra-hepatic cholangiocarcinoma: new insight into epidemiology and risk factors. World J Gastrointest Oncol. 2010;2(11):407–16.CrossRefGoogle Scholar
  16. 16.
    Weinbren K, Mutum SS. Pathological aspects of cholangiocarcinoma. J Pathol. 1983;139(2):217–38.CrossRefGoogle Scholar
  17. 17.
    Pitt HA, et al. Malignancies of the biliary tree. Curr Probl Surg. 1995;32(1):1–90.CrossRefGoogle Scholar
  18. 18.
    Jarnagin WR, et al. Papillary phenotype confers improved survival after resection of hilar cholangiocarcinoma. Ann Surg. 2005;241(5):703–12.CrossRefGoogle Scholar
  19. 19.
    Howe JR, et al. The American College of Surgeons Commission on Cancer and the American Cancer Society. Adenocarcinoma of the small bowel: review of the National Cancer Data Base, 1985–1995. Cancer. 1999;86(12):2693–706.CrossRefGoogle Scholar
  20. 20.
    Surveillance, Epidemiology, and End Results (SEER) Program. Research data (1973–2010), www.seer.cancer.gov. Accessed 18 Mar 2017.
  21. 21.
    Bilimoria KY, Bentrem DJ, Wayne JD, Ko CY, Bennett CL, Talamonti MS. Small bowel cancer in the United States: changes in epidemiology, treatment, and survival over the last 20 years. Ann Surg. 2009;249(1):63–71.CrossRefGoogle Scholar
  22. 22.
    Joesting DR, et al. Improving survival in adenocarcinoma of the duodenum. Am J Surg. 1981;141(2):228–31.CrossRefGoogle Scholar
  23. 23.
    Joensuu H, et al. Gastrointestinal stromal tumor (GIST). Ann Oncol. 2006;17(Suppl):280–6.CrossRefGoogle Scholar
  24. 24.
    Leese T, et al. Tumours and pseudotumours of the region of the ampulla of Vater: an endoscopic, clinical and pathological study. Gut. 1986;27(10):1186–92.CrossRefGoogle Scholar
  25. 25.
    Yamaguchi K, Enjoji M. Tsuneyoshi, pancreatoduodenal carcinoma: a clinicopathologic study of 304 patients and immunohistyochemical observation for CEA and CA19-9. J Surg Oncol. 1991;47(3):148–54.CrossRefGoogle Scholar
  26. 26.
    Talamini MA, et al. Adenocarcinoma of the ampulla of Vater. A 28-year experience. Ann Surg. 1997;225(5):590–9.CrossRefGoogle Scholar
  27. 27.
    Klempnauer J, et al. Carcinoma of the ampulla of Vater: determinants of long-term survival in 94 resected patients. HPB Surg. 1998;11(1):1–11.CrossRefGoogle Scholar
  28. 28.
    Beger HG, et al. Tumor of the ampulla of Vater: experience with local or radical resection in 171 consecutively treated patients. Arch Surg. 1999;134(5):526–32.CrossRefGoogle Scholar
  29. 29.
    Clary BM, et al. Local ampullary resection with careful intraoperative frozen section evaluation for presumed benign ampullary neoplasms. Surgery. 2000;127(6):628–33.CrossRefGoogle Scholar
  30. 30.
    Farnell MB, et al. Villous tumors of the duodenum: reappraisal of local vs. extended resection. J Gastrointest Surg. 2000;4(1):13–21, discussion 22–3.CrossRefGoogle Scholar
  31. 31.
    Rattner DW, et al. Defining the criteria for local resection of ampullary neoplasms. Arch Surg. 1996;131(4):366–71.CrossRefGoogle Scholar
  32. 32.
    Iwama T, et al. Indications for local excision of ampullary lesions associated with familial adenomatous polyposis. J Am Coll Surg. 1994;179(4):462–4.PubMedGoogle Scholar
  33. 33.
    Balthazar EJ, Chako AC. Computed tomography of pancreatic masses. Am J Gastroenterol. 1990;85(4):343–9.PubMedGoogle Scholar
  34. 34.
    Walsh RM, Connelly M, Baker M. Imaging for the diagnosis and staging of periampullary carcinomas. Surg Endosc. 2003;17(10):1514–20.CrossRefGoogle Scholar
  35. 35.
    Wyatt SH, Fishman EK. Spiral CT of the pancreas. Semin Ultrasound CT MR. 1994;15(2):122–32.CrossRefGoogle Scholar
  36. 36.
    Steiner E, et al. Imaging of pancreatic neoplasms: comparison of MR and CT. AJR Am J Roentgenol. 1989;152(3):487–91.CrossRefGoogle Scholar
  37. 37.
    Roggin KK, Yeh JJ, Ferrone CR, Riedel E, Gerdes H, Klimstra DS, Jaques DP, Brennan MF. Limitations of ampullectomy in the treatment of nonfamilial ampullary neoplasms. Ann Surg Oncol. 2005;12:971–80.CrossRefGoogle Scholar
  38. 38.
    Menzel J, Poremba C, Dietl KH, Böcker W, Domschke W. Tumors of the papilla of Vater – inadequate diagnostic impact of endoscopic forceps biopsies taken prior to and following sphincterotomy. Ann Oncol. 1999;10:1227–31.CrossRefGoogle Scholar
  39. 39.
    Elek G, Gyôri S, Tóth B, Pap A. Histological evaluation of preoperative biopsies from ampulla vateri. Pathol Oncol Res. 2003;9:32–41.CrossRefGoogle Scholar
  40. 40.
    Grobmyer SR, Stasik CN, Draganov P, Hemming AW, Dixon LR, Vogel SB, Hochwald SN. Contemporary results with ampullectomy for 29 “benign” neoplasms of the ampulla. J Am Coll Surg. 2008;206:466–71.CrossRefGoogle Scholar
  41. 41.
    Rivadeneira DE, et al. Comparison of linear array endoscopic ultrasound and helical computed tomography for the staging of periampullary malignancies. Ann Surg Oncol. 2003;10(8):890–7.CrossRefGoogle Scholar
  42. 42.
    Chen CH, Tseng LJ, Yang CC, Yeh YH. Preoperative evaluation of periampullary tumors by endoscopic sonography, transabdominal sonography, and computed tomography. J Clin Ultrasound. 2001;29:313–21.CrossRefGoogle Scholar
  43. 43.
    Kubo H, et al. Pre-operative staging of ampullary tumours by endoscopic ultrasound. Br J Radiol. 1999;72(857):443–7.CrossRefGoogle Scholar
  44. 44.
    Trikudanathan G, Njei B, Attam R, Arain M, Shaukat A. Staging accuracy of ampullary tumors by endoscopic ultrasound: meta-analysis and systematic review. Dig Endosc. 2014;26(5):617–26.CrossRefGoogle Scholar
  45. 45.
    Tierney WM, et al. The accuracy of EUS and helical CT in the assessment of vascular invasion by peripapillary malignancy. Gastrointest Endosc. 2001;53(2):182–8.CrossRefGoogle Scholar
  46. 46.
    Sharp KW, Brandes JL. Local resection of tumors of the ampulla of Vater. Am Surg. 1990;56(4):214–7.PubMedGoogle Scholar
  47. 47.
    Chen CH, Yang CC, Yeh YH, et al. Reappraisal of endosonography of ampullary tumors: correlation with transabdominal sonography, CT, and MRI. J Clin Ultrasound. 2009;37:18–25.CrossRefGoogle Scholar
  48. 48.
    Soriano A, et al. Preoperative staging and tumor resectability assessment of pancreatic cancer: prospective study comparing endoscopic ultrasonography, helical computed tomography, magnetic resonance imaging, and angiography. Am J Gastroenterol. 2004;99(3):492–501.CrossRefGoogle Scholar
  49. 49.
    Sun Y, et al. Single-operator cholangioscopy in the diagnosis of indeterminate biliary strictures. Gastrointest Endosc. 2015;82(6):1136–7.CrossRefGoogle Scholar
  50. 50.
    Allen VB, Gurusamy KS, Takwoingi Y, Kalia A, Davidson BR. Diagnostic accuracy of laparoscopy following computed tomography (CT) scanning for assessing the resectability with curative intent in pancreatic and periampullary cancer. Cochrane Database Syst Rev. 2016;7:CD009323.PubMedGoogle Scholar
  51. 51.
    Brooks AD, et al. The value of laparoscopy in the management of ampullary, duodenal, and distal bile duct tumors. J Gastrointest Surg. 2002;6(2):139–45.CrossRefGoogle Scholar
  52. 52.
    Beenen E, van Roest MHG, Sieders E, Peeters P, Porte RJ, de Boer MT, et al. Staging laparoscopy in patients scheduled for pancreaticoduodenectomy minimizes hospitalization in the remaining life time when metastatic carcinoma is found. Eur J Surg Oncol. 2014;40(8):989–94.CrossRefGoogle Scholar
  53. 53.
    Ridtitid W, et al. Endoscopic paillectomy: risk factors for incomplete resection and recurrence during long-term follow-up. Gastrointest Endosc. 2014;79(2):289–96.CrossRefGoogle Scholar
  54. 54.
    Clary BM, Pappas TN, Tyler DS. Transduodenal local resection for periampullary neoplasms. In: Evans DB, Abbruzzese JL, Pisters PW, editors. Pancreatic cancer, M.D. Anderson solid tumor oncology series. New York: Springer; 2007. p. 181–91.Google Scholar
  55. 55.
    Cahen DL, et al. Local resection or pancreaticoduodenectomy for villous adenoma of the ampulla of Vater diagnosed before operation. Br J Surg. 1997;84(7):948–51.CrossRefGoogle Scholar
  56. 56.
    Whipple AO, Parsons WB, Mullins CR. Treatment of carcinoma of the ampulla of Vater. Ann Surg. 1935;102(4):763–79.CrossRefGoogle Scholar
  57. 57.
    Yeo CJ, et al. Pancreaticoduodenectomy with or without distal gastrectomy and extended retroperitoneal lymphadenectomy for periampullary adenocarcinoma, part 2: randomized controlled trial evaluating survival, morbidity, and mortality. Ann Surg. 2002;236(3):355–66.CrossRefGoogle Scholar
  58. 58.
    Wray CJ, et al. Surgery for pancreatic cancer: recent controversies and current practice. Gastroenterology. 2005;128(6):1626–41.CrossRefGoogle Scholar
  59. 59.
    Schmidt CM, et al. Pancreaticoduodenectomy: a 20-year experience in 516 patients. Arch Surg. 2004;139(7):718–25.CrossRefGoogle Scholar
  60. 60.
    Stephens J, et al. Surgical morbidity, mortality, and long-term survival in patients with peripancreatic cancer following pancreaticoduodenectomy. Am J Surg. 1997;174(6):600–3.CrossRefGoogle Scholar
  61. 61.
    Klein P, et al. Is local excision of pT1-ampullary carcinomas justified? Eur J Surg Oncol. 1996;22(4):366–71.CrossRefGoogle Scholar
  62. 62.
    Branum GD, Pappas TN, Meyers WC. The management of tumors of the ampulla of Vater by local resection. Ann Surg. 1996;224(5):621–7.CrossRefGoogle Scholar
  63. 63.
    Su CH, et al. Factors affecting morbidity, mortality and survival after pancreaticoduodenectomy for carcinoma of the ampulla of Vater. Hepato-Gastroenterology. 1999;46(27):1973–9.PubMedGoogle Scholar
  64. 64.
    Howe JR, et al. Factors predictive of survival in ampullary carcinoma. Ann Surg. 1998;228(1):87–94.CrossRefGoogle Scholar
  65. 65.
    Cloyd JM, Norton JA, Visser BC, Poultsides GA. Does the extent of resection impact survival for duodenal adenocarcinoma? Analysis of 1,611 cases. Ann Surg Oncol. 2015;22:573–80.CrossRefGoogle Scholar
  66. 66.
    Onkendi EO, Boostrom SY, Sarr MG, et al. 15-year experience with surgical treatment of duodenal carcinoma: a comparison of periampullary and extra-ampullary duodenal carcinomas. J Gastrointest Surg. 2012;16(4):682–91.CrossRefGoogle Scholar
  67. 67.
    Cheng TY, et al. Effect of neoadjuvant chemoradiation on operative mortality and morbidity for pancreaticoduodenectomy. Ann Surg Oncol. 2006;13(1):66–74.CrossRefGoogle Scholar
  68. 68.
    Ferrone CR, et al. Tadiological and surgical implications of neoadjuvant treatment with FOLFIRINOX for locally advanced and borderline resectable pancreatic cancer. Ann Surg. 2015;261(1):12–7.CrossRefGoogle Scholar
  69. 69.
    Mehta VK, et al. Adjuvant chemoradiotherapy for “unfavorable” carcinoma of the ampulla of Vater: preliminary report. Arch Surg. 2001;136(1):65–9.CrossRefGoogle Scholar
  70. 70.
    Smeenk HG, et al. Long-term survival and metastatic pattern of pancreatic and periampullary cancer after adjuvant chemoradiation or observation: long-term results of EORTC trial 40891. Ann Surg. 2007;246(5):734–40.CrossRefGoogle Scholar
  71. 71.
    Kwon J, Kim BH, Kim K, et al. Survival benefit of adjuvant chemoradiotherapy in patients with ampulla of Vater cancer. Ann Surg. 2015;262(1):47–52.CrossRefGoogle Scholar
  72. 72.
    Takada T, et al. Is postoperative adjuvant chemotherapy useful for gallbladder carcinoma? A phase III multicenter prospective randomized controlled trial in patients with resected pancreaticobiliary carcinoma. Cancer. 2002;95(8):1685–95.CrossRefGoogle Scholar
  73. 73.
    Hughes MA, et al. Adjuvant concurrent chemoradiation for adenocarcinoma of the distal common bile duct. Int J Radiat Oncol Biol Phys. 2007;68(1):178–82.CrossRefGoogle Scholar
  74. 74.
    Ben-Josef E, Guthrie KA, El-Khoueiry AB, et al. SWOG S0809: a phase II intergroup trial of adjuvant capecitabine and gemcitabine followed by radiotherapy and concurrent capecitabine in extrahepatic cholangiocarcinoma and gallbladder carcinoma. J Clin Oncol. 2015;33(24):2617–22.CrossRefGoogle Scholar
  75. 75.
    Swartz MJ, et al. Adjuvant concurrent chemoradiation for node-positive adenocarcinoma of the duodenum. Arch Surg. 2007;142(3):285–8.CrossRefGoogle Scholar
  76. 76.
    Neoptolemos JP, et al. European Study Group for Pancreatic Cancer. Effect of adjuvant chemotherapy with flourouracil plus folinic acid or gemcitabine vs observation on survival in patients with resected periampullary adenocarcinoma. The ESPAC-3 Periampullary Cancer Randomized Trial. JAMA. 2012;308(2):147–56.CrossRefGoogle Scholar
  77. 77.
    Yeo CJ, et al. Periampullary adenocarcinoma. Analysis of 5-year survivors. Ann Surg. 1998;227(6):821–31.CrossRefGoogle Scholar
  78. 78.
    Fong Y, et al. Outcome of treatment for distal bile duct cancer. Br J Surg. 1996;83(12):1712–5.CrossRefGoogle Scholar
  79. 79.
    Wade TP, et al. Experience with distal bile duct cancers in U.S. Veterans Affairs hospitals: 1987–1991. J Surg Oncol. 1997;64(3):242–5.CrossRefGoogle Scholar
  80. 80.
    Jang J-Y, Kim S-W, Park DJ, et al. Actual long-term outcome of extrahepatic bile duct cancer after surgical resection. Ann Surg. 2005;241(1):77–84.PubMedPubMedCentralGoogle Scholar
  81. 81.
    DeOliveira ML, et al. Cholangiocarcinoma: thirty-one-year experience with 564 patients at a single institution. Ann Surg. 2007;245(5):755–62.CrossRefGoogle Scholar
  82. 82.
    Nagakawa T, et al. Biliary tract cancer treatment: results from the Biliary Tract Cancer Statistics Registry in Japan. J Hepato-Biliary-Pancreat Surg. 2002;9(5):569–75.CrossRefGoogle Scholar
  83. 83.
    Bakaeen FG, et al. What prognostic factors are important in duodenal adenocarcinoma? Arch Surg. 2000;135(6):635–41.CrossRefGoogle Scholar
  84. 84.
    Sarela AI, et al. Adenocarcinoma of the duodenum: importance of accurate lymph node staging and similarity in outcome to gastric cancer. Ann Surg Oncol. 2004;11(4):380–6.CrossRefGoogle Scholar
  85. 85.
    Sohn TA, et al. Adenocarcinoma of the duodenum: factors influencing long-term survival. J Gastrointest Surg. 1998;2(1):79–87.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Heather A. Lillemoe
    • 1
  • John D. Abad
    • 2
  • Keith D. Lillemoe
    • 3
  1. 1.Vanderbilt University Medical CenterNashvilleUSA
  2. 2.Feinberg School of MedicineNorthwestern UniversityWarrenvilleUSA
  3. 3.Massachusetts General Hospital, Harvard Medical SchoolBostonUSA

Personalised recommendations