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Ampullary adenomas: Clinical manifestations and diagnosis

Author
John A Martin, MD
Section Editor
Lawrence S Friedman, MD
Deputy Editor
Shilpa Grover, MD, MPH, AGAF

INTRODUCTION

Benign neoplasms of the ampulla of Vater are rare, representing less than 10 percent of periampullary neoplasms [1,2]. Adenomas are the most common benign lesions of the ampulla but have the potential to undergo malignant transformation to ampullary carcinomas [1,3-18]. This topic will review the clinical manifestations and diagnosis of ampullary adenomas. The management of ampullary adenomas and the clinical presentation, diagnosis, and management of ampullary cancer are discussed elsewhere. (See "Ampullary adenomas: Management" and "Ampullary carcinoma: Epidemiology, clinical manifestations, diagnosis and staging" and "Ampullary carcinoma: Treatment and prognosis".)

EPIDEMIOLOGY

Ampullary adenomas are detected in 0.04 to 0.12 percent of individuals in autopsy series [8,19,20]. Ampullary adenomas can occur sporadically, or in the setting of an adenomatous polyposis syndromes, including familial adenomatous polyposis and MUTYH-associated polyposis. Sporadic ampullary adenomas are usually diagnosed in patients older than 40 years of age and most commonly in their seventies. In contrast, patients with ampullary adenomas in the setting of an adenomatous polyposis syndrome are frequently diagnosed decades earlier in the setting of a surveillance program rather than because of symptoms [21]. (See "Clinical manifestations and diagnosis of familial adenomatous polyposis", section on 'Extracolonic manifestations' and "MUTYH-associated polyposis", section on 'Extracolonic manifestations'.)

CLINICAL FEATURES

Clinical features are a consequence of a mass-effect of the adenoma compressing and impeding biliary or pancreatic outflow.

Clinical presentation — Jaundice is the most common presenting symptom and is present in 50 to 75 percent of patients [3,22-25]. It is usually painless or accompanied by a vague or dull ache in the epigastrium [3]. Patients rarely have pruritus and infrequently develop cholangitis [26]. Other symptoms include biliary colic, nausea, and vomiting [3,24,27]. Less commonly, patients can present with acute pancreatitis, iron deficiency anemia, and overt upper gastrointestinal bleeding [22,27-31].

Imaging features — Patients with ampullary adenomas usually have evidence of biliary ductal dilation on imaging of the abdomen performed for evaluation of obstructive jaundice. Up to 25 percent of patients have associated common bile duct stones secondary to cholestasis [32]. Abdominal imaging is not diagnostic for an ampullary adenomas as it does not permit direct luminal visualization of the papillary aspect of the ampulla to provide access for tissue acquisition for histopathology. (See 'Diagnosis' below and "Diagnostic approach to the adult with jaundice or asymptomatic hyperbilirubinemia", section on 'Suspected biliary obstruction or intrahepatic cholestasis'.)

       
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Literature review current through: Sep 2017. | This topic last updated: Sep 19, 2017.
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References
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  1. Park SH, Kim YI, Park YH, et al. Clinicopathologic correlation of p53 protein overexpression in adenoma and carcinoma of the ampulla of Vater. World J Surg 2000; 24:54.
  2. Park SW, Song SY, Chung JB, et al. Endoscopic snare resection for tumors of the ampulla of Vater. Yonsei Med J 2000; 41:213.
  3. Treitschke F, Beger HG. Local resection of benign periampullary tumors. Ann Oncol 1999; 10 Suppl 4:212.
  4. Allgaier HP, Schwacha H, Kleinschmidt M, et al. Ampullary hamartoma: A rare cause of biliary obstruction. Digestion 1999; 60:497.
  5. Deschamps L, Dokmak S, Guedj N, et al. Mixed endocrine somatostatinoma of the ampulla of vater associated with a neurofibromatosis type 1: a case report and review of the literature. JOP 2010; 11:64.
  6. Sun JH, Chao M, Zhang SZ, et al. Coexistence of small cell neuroendocrine carcinoma and villous adenoma in the ampulla of Vater. World J Gastroenterol 2008; 14:4709.
  7. Kwon TH, Park DH, Shim KY, et al. Ampullary adenomyoma presenting as acute recurrent pancreatitis. World J Gastroenterol 2007; 13:2892.
  8. Sato T, Konishi K, Kimura H, et al. Adenoma and tiny carcinoma in adenoma of the papilla of Vater--p53 and PCNA. Hepatogastroenterology 1999; 46:1959.
  9. Sellner FJ, Riegler FM, Machacek E. Implications of histological grade of tumour for the prognosis of radically resected periampullary adenocarcinoma. Eur J Surg 1999; 165:865.
  10. Stolte M, Pscherer C. Adenoma-carcinoma sequence in the papilla of Vater. Scand J Gastroenterol 1996; 31:376.
  11. Asbun HJ, Rossi RL, Munson JL. Local resection for ampullary tumors. Is there a place for it? Arch Surg 1993; 128:515.
  12. Seifert E, Schulte F, Stolte M. Adenoma and carcinoma of the duodenum and papilla of Vater: a clinicopathologic study. Am J Gastroenterol 1992; 87:37.
  13. Yamaguchi K, Enjoji M. Adenoma of the ampulla of Vater: putative precancerous lesion. Gut 1991; 32:1558.
  14. Ryan DP, Schapiro RH, Warshaw AL. Villous tumors of the duodenum. Ann Surg 1986; 203:301.
  15. Kozuka S, Tsubone M, Yamaguchi A, Hachisuka K. Adenomatous residue in cancerous papilla of Vater. Gut 1981; 22:1031.
  16. Perzin KH, Bridge MF. Adenomas of the small intestine: a clinicopathologic review of 51 cases and a study of their relationship to carcinoma. Cancer 1981; 48:799.
  17. Ruemmele P, Dietmaier W, Terracciano L, et al. Histopathologic features and microsatellite instability of cancers of the papilla of vater and their precursor lesions. Am J Surg Pathol 2009; 33:691.
  18. Wagner PL, Chen YT, Yantiss RK. Immunohistochemical and molecular features of sporadic and FAP-associated duodenal adenomas of the ampullary and nonampullary mucosa. Am J Surg Pathol 2008; 32:1388.
  19. Shapiro PF, Lifvendahl RA. Tumors of the Extrahepatic Bile-ducts. Ann Surg 1931; 94:61.
  20. BAKER HL, CALDWELL DW. Lesions of the ampulla of Vater. Surgery 1947; 21:523.
  21. Bleau BL, Gostout CJ. Endoscopic treatment of ampullary adenomas in familial adenomatous polyposis. J Clin Gastroenterol 1996; 22:237.
  22. Ashkar K, Deeb LS, Bikhazi K, Arnaout MS. Unusual manifestation of an ampullary tumor presenting with severe upper gastrointestinal bleeding. Digestion 1999; 60:583.
  23. Sharp KW, Brandes JL. Local resection of tumors of the ampulla of Vater. Am Surg 1990; 56:214.
  24. Taxier M, Sivak MV Jr, Cooperman A. Villous adenoma of the ampulla of Vater. Gastrointest Endosc 1979; 25:155.
  25. Greco S, Cassinotti A, Massari A, et al. Isolated ampullary adenoma causing biliary obstruction. J Gastrointestin Liver Dis 2008; 17:329.
  26. Sand JA, Nordback IH. Transduodenal excision of benign adenoma of the papilla of Vater. Eur J Surg 1995; 161:269.
  27. Shemesh E, Nass S, Czerniak A. Endoscopic sphincterotomy and endoscopic fulguration in the management of adenoma of the papilla of Vater. Surg Gynecol Obstet 1989; 169:445.
  28. Sato T, Konishi K, Kimura H, et al. Necrotizing acute pancreatitis caused by tiny carcinoma in adenoma in Vater's papilla. Gastrointest Endosc 1999; 50:672.
  29. Ohmori K, Kinoshita H, Shiraha Y, Satake K. Pancreatic duct obstruction by a benign polypoid adenoma of the ampulla of Vater. Am J Surg 1976; 132:662.
  30. Akatsu T, Aiura K, Takahashi S, et al. Recurrent pancreatitis caused by ampullary carcinoma and minor papilla adenoma in familial polyposis: report of a case. Surg Today 2008; 38:440.
  31. Murakami Y, Uemura K, Hayashidani Y, et al. Relapsing acute pancreatitis due to ampullary adenoma in a patient with familial adenomatous polyposis. J Gastroenterol 2006; 41:798.
  32. Binmoeller KF, Boaventura S, Ramsperger K, Soehendra N. Endoscopic snare excision of benign adenomas of the papilla of Vater. Gastrointest Endosc 1993; 39:127.
  33. Skordilis P, Mouzas IA, Dimoulios PD, et al. Is endosonography an effective method for detection and local staging of the ampullary carcinoma? A prospective study. BMC Surg 2002; 2:1.
  34. Qiao QL, Zhao YG, Ye ML, et al. Carcinoma of the ampulla of Vater: factors influencing long-term survival of 127 patients with resection. World J Surg 2007; 31:137.
  35. Lee M, Kim MJ, Park MS, et al. Using multi-detector-row CT to diagnose ampullary adenoma or adenocarcinoma in situ. Eur J Radiol 2011; 80:e340.
  36. Bakkevold KE, Arnesjø B, Kambestad B. Carcinoma of the pancreas and papilla of Vater: presenting symptoms, signs, and diagnosis related to stage and tumour site. A prospective multicentre trial in 472 patients. Norwegian Pancreatic Cancer Trial. Scand J Gastroenterol 1992; 27:317.
  37. Ponchon T, Berger F, Chavaillon A, et al. Contribution of endoscopy to diagnosis and treatment of tumors of the ampulla of Vater. Cancer 1989; 64:161.
  38. Bourgeois N, Dunham F, Verhest A, Cremer M. Endoscopic biopsies of the papilla of Vater at the time of endoscopic sphincterotomy: difficulties in interpretation. Gastrointest Endosc 1984; 30:163.
  39. Gincul R, Ciocirlan M, Dumortier J, et al. Severe acute pancreatitis following endoscopic biopsy of the minor duodenal papilla. Endoscopy 2009; 41 Suppl 2:E195.
  40. 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; 59:506.
  41. Posner S, Colletti L, Knol J, et al. Safety and long-term efficacy of transduodenal excision for tumors of the ampulla of Vater. Surgery 2000; 128:694.
  42. Clary BM, Tyler DS, Dematos P, et al. Local ampullary resection with careful intraoperative frozen section evaluation for presumed benign ampullary neoplasms. Surgery 2000; 127:628.
  43. Takashima M, Ueki T, Nagai E, et al. Carcinoma of the ampulla of Vater associated with or without adenoma: a clinicopathologic analysis of 198 cases with reference to p53 and Ki-67 immunohistochemical expressions. Mod Pathol 2000; 13:1300.
  44. Beger HG, Treitschke F, Gansauge F, et al. Tumor of the ampulla of Vater: experience with local or radical resection in 171 consecutively treated patients. Arch Surg 1999; 134:526.
  45. Yamaguchi K, Enjoji M, Kitamura K. Endoscopic biopsy has limited accuracy in diagnosis of ampullary tumors. Gastrointest Endosc 1990; 36:588.
  46. Lee SY, Jang KT, Lee KT, et al. Can endoscopic resection be applied for early stage ampulla of Vater cancer? Gastrointest Endosc 2006; 63:783.
  47. Younes M, Riley S, Genta RM, et al. p53 protein accumulation in tumors of the ampulla of Vater. Cancer 1995; 76:1150.
  48. Howe JR, Klimstra DS, Cordon-Cardo C, et al. K-ras mutation in adenomas and carcinomas of the ampulla of vater. Clin Cancer Res 1997; 3:129.
  49. Chung CH, Wilentz RE, Polak MM, et al. Clinical significance of K-ras oncogene activation in ampullary neoplasms. J Clin Pathol 1996; 49:460.
  50. Murray MA, Zimmerman MJ, Ee HC. Sporadic duodenal adenoma is associated with colorectal neoplasia. Gut 2004; 53:261.
  51. Apel D, Jakobs R, Spiethoff A, Riemann JF. Follow-up after endoscopic snare resection of duodenal adenomas. Endoscopy 2005; 37:444.