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Miscellaneous Non-Pancreatic Non-Endocrine Tumors

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Pancreatic Cancer
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Abstract:

Non-endocrine, non-pancreatic 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, 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 non-pancreatic 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 non-pancreatic and non-neuroendocrine periampullary tumors and will include the pathology, clinical presentation, diagnostic work-up, and management strategies to approach these neoplasms.

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References

  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):1191–1204.

    Article  PubMed  Google Scholar 

  2. Riall TS, et al.: Resected periampullary adenocarcinoma: 5-year survivors and their 6- to 10-year follow-up. Surgery 2006;140(5):764–772.

    Article  PubMed  Google Scholar 

  3. Stolte M, Pscherer C: Adenoma-carcinoma sequence in the papilla of Vater. Scand J Gastroenterol 1996;31(4):376–382.

    Article  CAS  PubMed  Google Scholar 

  4. Galandiuk S, et al.: Villous tumors of the duodenum. Ann Surg 1988;207(3):234–239.

    Article  CAS  PubMed  Google Scholar 

  5. Rosenberg J, et al.: Benign villous adenomas of the ampulla of Vater. Cancer 1986;58(7):1563–1568.

    Article  CAS  PubMed  Google Scholar 

  6. Roder JD, et al.: Number of lymph node metastases is significantly associated with survival in patients with radically resected carcinoma of the ampulla of Vater. Br J Surg 1995;82(12):1693–1696.

    Article  CAS  PubMed  Google Scholar 

  7. Blackman E, Nash SV: Diagnosis of duodenal and ampullary epithelial neoplasms by endoscopic biopsy: a clinicopathologic and immunohistochemical study. Hum Pathol 1985;16(9):901–910.

    Article  CAS  PubMed  Google Scholar 

  8. 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):506–515.

    Article  CAS  PubMed  Google Scholar 

  9. Noda Y, et al.: Histologic follow-up of ampullary adenomas in patients with familial adenomatosis coli. Cancer 1992;70(7):1847–1856.

    Article  CAS  PubMed  Google Scholar 

  10. Bjork J, et al.: Periampullary adenomas and adenocarcinomas in familial adenomatous polyposis: cumulative risks and APC gene mutations. Gastroenterology 2001;121(5):1127–1135.

    Article  CAS  PubMed  Google Scholar 

  11. Wittekind C, Tannapfel A: Adenoma of the papilla and ampulla – premalignant lesions? Langenbecks Arch Surg 2001;386(3):172–175.

    Article  CAS  PubMed  Google Scholar 

  12. Iwama T, et al.: Indications for local excision of ampullary lesions associated with familial adenomatous polyposis. J Am Coll Surg 1994;179(4):462–464.

    CAS  PubMed  Google Scholar 

  13. Spigelman AD, et al.: Evidence for adenoma-carcinoma sequence in the duodenum of patients with familial adenomatous polyposis. The Leeds Castle Polyposis Group (Upper Gastrointestinal Committee). J Clin Pathol 1994;47(8):709–710.

    Article  CAS  PubMed  Google Scholar 

  14. Mino M, Lauwers GY: Pathology of periampullary tumors. In: Von Hoff DD, Evans DB, Hruban RH, (eds.): Pancreatic Cancer. Jones and Bartlett, 2005: 686–702.

    Google Scholar 

  15. Carter JT, et al.: Tumors of the ampulla of Vater: histopathologic classification and predictors of survival. J Am Coll Surg 2008;207(2):210–218.

    Article  PubMed  Google Scholar 

  16. Komorowski RA, et al.: Assessment of ampulla of Vater pathology. An endoscopic approach. Am J Surg Pathol 1991;15(12):1188–1196.

    Article  CAS  PubMed  Google Scholar 

  17. 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):1078–1091.

    Article  CAS  PubMed  Google Scholar 

  18. Fong Y, et al.: Outcome of treatment for distal bile duct cancer. Br J Surg 1996;83(12):1712–1715.

    Article  CAS  PubMed  Google Scholar 

  19. Mir-Madjlessi SH, Farmer RG, Sivak MV Jr: Bile duct carcinoma in patients with ulcerative colitis. Relationship to sclerosing cholangitis: report of six cases and review of the literature. Dig Dis Sci 1987;32(2):145–154.

    Article  CAS  PubMed  Google Scholar 

  20. Bergquist A, et al.: Hepatic and extrahepatic malignancies in primary sclerosing cholangitis. J Hepatol 2002;36(3):321–327.

    Article  PubMed  Google Scholar 

  21. Nakeeb A, et al.: Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors. Ann Surg 1996;224(4):463–473.

    Article  CAS  PubMed  Google Scholar 

  22. Wade TP, et al.: Experience with distal bile duct cancers in U.S. Veterans Affairs hospitals: 1987–1991. J Surg Oncol 1997;64(3):242–245.

    Article  CAS  PubMed  Google Scholar 

  23. Yeo CJ, et al.: Six hundred fifty consecutive pancreaticoduodenectomies in the 1990s: pathology, complications, and outcomes. Ann Surg 1997;226(3):248–257.

    Article  CAS  PubMed  Google Scholar 

  24. Weinbren K, Mutum SS: Pathological aspects of cholangiocarcinoma. J Pathol 1983;139(2):217–238.

    Article  CAS  PubMed  Google Scholar 

  25. Pitt HA, et al.: Malignancies of the biliary tree. Curr Probl Surg 1995;32(1):1–90.

    Article  CAS  PubMed  Google Scholar 

  26. Jarnagin WR, et al.: Papillary phenotype confers improved survival after resection of hilar cholangiocarcinoma. Ann Surg 2005;241(5):703–712.

    Article  PubMed  Google Scholar 

  27. 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):2693–2706.

    Article  CAS  PubMed  Google Scholar 

  28. Jemal A, et al.: Cancer statistics, 2003. CA Cancer J Clin 2003;53(1):5–26.

    Article  PubMed  Google Scholar 

  29. Joesting DR, et al.: Improving survival in adenocarcinoma of the duodenum. Am J Surg 1981;141(2):228–231.

    Article  CAS  PubMed  Google Scholar 

  30. 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):1186–1192.

    Article  CAS  PubMed  Google Scholar 

  31. Yamaguchi K, Enjoji M: Tsuneyoshi, Pancreatoduodenal carcinoma: a clinicopathologic study of 304 patients and immunohistochemical observation for CEA and CA19–9. J Surg Oncol 1991;47(3):148–154.

    Article  CAS  PubMed  Google Scholar 

  32. Talamini MA, et al.: Adenocarcinoma of the ampulla of Vater. A 28-year experience. Ann Surg 1997;225(5):590–599.

    Article  CAS  PubMed  Google Scholar 

  33. 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.

    Article  CAS  PubMed  Google Scholar 

  34. 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):526–532.

    Article  CAS  PubMed  Google Scholar 

  35. Clary BM, et al.: Local ampullary resection with careful intraoperative frozen section evaluation for presumed benign ampullary neoplasms. Surgery 2000;127(6):628–633.

    Article  CAS  PubMed  Google Scholar 

  36. Farnell MB, et al.: Villous tumors of the duodenum: reappraisal of local vs. extended resection. J Gastrointest Surg 2000;4(1):13–21, discussion 22–23.

    Article  CAS  PubMed  Google Scholar 

  37. Rattner DW, et al.: Defining the criteria for local resection of ampullary neoplasms. Arch Surg 1996;131(4):366–371.

    CAS  PubMed  Google Scholar 

  38. Balthazar EJ, Chako AC: Computed tomography of pancreatic masses. Am J Gastroenterol 1990;85(4):343–349.

    CAS  PubMed  Google Scholar 

  39. Walsh RM, Connelly M, Baker M: Imaging for the diagnosis and staging of periampullary carcinomas. Surg Endosc 2003;17(10):1514–1520.

    Article  CAS  PubMed  Google Scholar 

  40. Wyatt SH, Fishman EK: Spiral CT of the pancreas. Semin Ultrasound CT MR 1994;15(2):122–132.

    Article  CAS  PubMed  Google Scholar 

  41. Legmann P, et al.: Pancreatic tumors: comparison of dual-phase helical CT and endoscopic sonography. AJR Am J Roentgenol 1998;170(5):1315–1322.

    CAS  PubMed  Google Scholar 

  42. Steiner E, et al.: Imaging of pancreatic neoplasms: comparison of MR and CT. AJR Am J Roentgenol 1989;152(3):487–491.

    CAS  PubMed  Google Scholar 

  43. Ross WA, Bismar MM: Evaluation and management of periampullary tumors. Curr Gastroenterol Rep 2004;6(5):362–370.

    Article  PubMed  Google Scholar 

  44. 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):890–897.

    Article  PubMed  Google Scholar 

  45. Kubo H, et al.: Pre-operative staging of ampullary tumours by endoscopic ultrasound. Br J Radiol 1999;72(857):443–447.

    CAS  PubMed  Google Scholar 

  46. 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):182–188.

    Article  CAS  PubMed  Google Scholar 

  47. Sharp KW, Brandes JL: Local resection of tumors of the ampulla of Vater. Am Surg 1990;56(4):214–217.

    CAS  PubMed  Google Scholar 

  48. Mukai H, et al.: Evaluation of endoscopic ultrasonography in the pre-operative staging of carcinoma of the ampulla of Vater and common bile duct. Gastrointest Endosc 1992;38(6):676–683.

    Article  CAS  PubMed  Google Scholar 

  49. Souquet JC, et al.: Endosonography-guided treatment of esophageal carcinoma. Endoscopy 1992;24(Suppl. 1):324–328.

    Article  PubMed  Google Scholar 

  50. 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.

    Article  PubMed  Google Scholar 

  51. Warshaw AL, Tepper JE, Shipley WU: Laparoscopy in the staging and planning of therapy for pancreatic cancer. Am J Surg 1986;151(1):76–80.

    Article  CAS  PubMed  Google Scholar 

  52. Friess H, et al.: The role of diagnostic laparoscopy in pancreatic and periampullary malignancies. J Am Coll Surg 1998;186(6):675–682.

    Article  CAS  PubMed  Google Scholar 

  53. 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):139–145.

    Article  PubMed  Google Scholar 

  54. Clary BM, Pappas TN, Tyler DS. Transduodenal local resection for periampullary neoplasms. In: Evans DB, Abbruzzese JL, Pisters PW, (eds.): Pancreatic Cancer: M.D. Anderson Solid Tumor Oncology Series. New York: Springer-Verlag; 2007: 181–191.

    Google Scholar 

  55. Posner S, et al.: Safety and long-term efficacy of transduodenal excision for tumors of the ampulla of Vater. Surgery 2000;128(4):694–701.

    Article  CAS  PubMed  Google Scholar 

  56. 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):948–951.

    Article  CAS  PubMed  Google Scholar 

  57. Whipple AO, Parsons WB, Mullins CR: Treatment of Carcinoma of the Ampulla of Vater. Ann Surg 1935;102(4):763–779.

    Article  CAS  PubMed  Google Scholar 

  58. 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):355–366.

    Article  PubMed  Google Scholar 

  59. Wray CJ, et al.: Surgery for pancreatic cancer: recent controversies and current practice. Gastroenterology 2005;128(6):1626–1641.

    Article  PubMed  Google Scholar 

  60. Schmidt CM, et al.: Pancreaticoduodenectomy: a 20-year experience in 516 patients. Arch Surg 2004;139(7):718–725.

    Article  PubMed  Google Scholar 

  61. Stephens J, et al.: Surgical morbidity, mortality, and long-term survival in patients with peripancreatic cancer following pancreaticoduodenectomy. Am J Surg 1997;174(6):600–603.

    Article  CAS  PubMed  Google Scholar 

  62. Klein P, et al.: Is local excision of pT1-ampullary carcinomas justified?. Eur J Surg Oncol 1996;22(4):366–371.

    Article  CAS  PubMed  Google Scholar 

  63. Branum GD, Pappas TN, Meyers WC: The management of tumors of the ampulla of Vater by local resection. Ann Surg 1996;224(5):621–627.

    Article  CAS  PubMed  Google Scholar 

  64. Su CH, et al.: Factors affecting morbidity, mortality and survival after pancreaticoduodenectomy for carcinoma of the ampulla of Vater. Hepatogastroenterology 1999;46(27):1973–1979.

    CAS  PubMed  Google Scholar 

  65. Roberts RH, et al.: Pancreaticoduodenectomy of ampullary carcinoma. Am Surg 1999;65(11):1043–1048.

    CAS  PubMed  Google Scholar 

  66. Howe JR, et al.: Factors predictive of survival in ampullary carcinoma. Ann Surg 1998;228(1):87–94.

    Article  CAS  PubMed  Google Scholar 

  67. Cheng TY, et al.: Effect of neoadjuvant chemoradiation on operative mortality and morbidity for pancreaticoduodenectomy. Ann Surg Oncol 2006;13(1):66–74.

    Article  PubMed  Google Scholar 

  68. Mehta VK, et al.: Adjuvant chemoradiotherapy for “unfavorable” carcinoma of the ampulla of Vater: preliminary report. Arch Surg 2001;136(1):65–69.

    Article  CAS  PubMed  Google Scholar 

  69. 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):734–740.

    Article  PubMed  Google Scholar 

  70. 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):1685–1695.

    Article  PubMed  Google Scholar 

  71. Hughes MA, et al.: Adjuvant concurrent chemoradiation for adenocarcinoma of the distal common bile duct. Int J Radiat Oncol Biol Phys 2007;68(1):178–182.

    Article  CAS  PubMed  Google Scholar 

  72. Swartz MJ, et al.: Adjuvant concurrent chemoradiation for node-positive adenocarcinoma of the duodenum. Arch Surg 2007;142(3):285–288.

    Article  PubMed  Google Scholar 

  73. Yeo CJ, etal.: Periampullary adenocarcinoma. Analysis of 5-year survivors. Ann Surg 1998;227(6): 821–831.

    Google Scholar 

  74. Nagorney DM, et al.: Outcomes after curative resections of cholangiocarcinoma. Arch Surg 1993;128(8):871–877.

    CAS  PubMed  Google Scholar 

  75. DeOliveira ML, et al.: Cholangiocarcinoma: thirty-one-year experience with 564 patients at a single institution. Ann Surg 2007;245(5):755–762.

    Article  PubMed  Google Scholar 

  76. Nagakawa T, et al.: Biliary tract cancer treatment: results from the Biliary Tract Cancer Statistics Registry in Japan. J Hepatobiliary Pancreat Surg 2002;9(5):569–575.

    Article  PubMed  Google Scholar 

  77. Bakaeen FG, et al.: What prognostic factors are important in duodenal adenocarcinoma? Arch Surg 2000;135(6):635–641.

    Article  CAS  PubMed  Google Scholar 

  78. 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):380–386.

    Article  PubMed  Google Scholar 

  79. Sohn TA, et al.: Adenocarcinoma of the duodenum: factors influencing long-term survival. J Gastrointest Surg 1998;2(1):79–87.

    Article  CAS  PubMed  Google Scholar 

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Abad, J.D., Lillemoe, K.D. (2010). Miscellaneous Non-Pancreatic Non-Endocrine Tumors. In: Pancreatic Cancer. Springer, New York, NY. https://doi.org/10.1007/978-0-387-77498-5_10

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