eMedicine Specialties > Oncology > Carcinomas of the Gastrointestinal Tract

Carcinoma of the Ampulla of Vater

Author: Nafisa K Kuwajerwala, MD, Staff Surgeon, Breast Oncology, William Beaumont Hospital
Coauthor(s): Pankaj Chaturvedi, MBBS, MS, Associate Professor, Head and Neck Surgery, Department of Surgical Oncology, Tata Memorial Hospital, India; Ronald S Chamberlain, MD, Chairman, Chief, Department of Surgery, Saint Barnabas Medical Center; Venkata Subramanian Kanthimathinathan, MD, Staff Physician, Department of General Surgery, Loma Linda University Medical Center; Uma Chaturvedi, MD, MBBS, DPB, Lecturer, Department of Pathology, KJ Somaiya Hospital and Research Center, India; Gunateet Goswami, MD, Consulting Staff, Internal Medicine Associates, Mount Clemens, Michigan; Consulting Staff, Department of Cardiology, Henry Ford Hospital
Contributor Information and Disclosures

Updated: Apr 8, 2009

Introduction

Background

Carcinoma of the ampulla of Vater is a malignant tumor arising within 2 cm of the distal end of the common bile duct, where it passes through the wall of the duodenum and ampullary papilla. The common bile duct merges with the pancreatic duct of Wirsung at this point and exits through the ampulla into the duodenum. The most distal portion of the common bile duct is dilated (ie, forms the ampulla of Vater) and is surrounded by the sphincter of Oddi, which spirals upward around the terminal portion of the duct. Because of biliary outflow obstruction, carcinoma of the ampulla of Vater tends to manifest early, as opposed to other pancreatic neoplasms that often are advanced at the time of diagnosis.

Curative surgical resection is the only option for long-term survival. Surgical or radiologic biliary decompression, relief of gastric outlet obstruction, and adequate pain control may improve the quality of life but do not affect overall survival rate.

Pathophysiology

Ninety percent of ampullary tumors are adenocarcinomas. Neuroendocrine tumors, cystadenomas, and adenomas represent additional, but uncommon, histologic types. Tumors originate from ductal epithelial cells and usually invade into the substance of the pancreas. In more advanced disease states, peripancreatic tissue and the adventitia of large neighboring vessels, such as the superior mesenteric and portal veins, may be involved.

Lymph nodes metastases are present in as many as half of patients. Pericanalicular lymph nodes usually are the first to be involved. Nodes along the superior mesenteric, gastroduodenal, common hepatic, and splenic arteries, as well as the celiac trunk, are the second station of lymph nodes. Perineural, vascular, and lymphatic invasion are associated with a poor prognosis. Liver is the most common site (66%) of distant metastasis, followed by lymph nodes (22%). In advanced cases, lung metastasis also may occur.

Frequency

United States

Carcinoma of the ampulla of Vater is an uncommon tumor; fewer than 2000 cases are diagnosed per year. Ampullary cancer accounts for approximately 0.2% of all gastrointestinal tract malignancies and about 7% of all periampullary carcinomas. Adenocarcinoma of the ampulla of Vater is the second most common periampullary malignancy.

International

Worldwide incidence is not known.

Mortality/Morbidity

  • Most of these tumors are resectable for cure at diagnosis; however, the 5-year survival rate is only 40%.
  • Operative mortality rates have decreased significantly over the last decade because of increased surgical experience, improved anesthesia, better preoperative imaging, and better postoperative management.
  • Pancreatic fistulas, prolonged gastric emptying, wound complications, intraabdominal sepsis, thrombophlebitis, and marginal ulceration are the most common complications.
  • Postoperative mortality rates in the best centers are 2-5%.

Race

  • No race predilection is seen.

Sex

  • No sex predilection is seen.

Age

  • Ampullary cancer most often is seen in the fifth through the seventh decades of life.

Clinical

History

  • Jaundice
    • Jaundice is the presenting symptom in three fourths of cases. Ampullary cancer has no additional classic early symptoms.
    • Jaundice may intermittently wax and wane because of central necrosis and sloughing or pressure opening of a minimally obstructed duct.
  • Other features
    • Pruritus
    • Loss of appetite
    • Dyspepsia and vomiting: These may be present if the duodenal lumen is compromised.
    • Progressive weight loss
    • Epigastric pain: The abdominal pain usually is dull, aching midepigastric pain or right hypochondriac pain. Backache is usually a sign of advanced stage.
    • Diarrhea may occur with this tumor  due to the absence of lipase within the gut related to pancreatic duct obstruction.
    • Hematemesis, melena, and hematochezia: These are uncommon features caused by tumor bleeding.

Physical

  • The Courvoisier sign, painless jaundice associated with a palpable gallbladder, may be present. Unlike that due to a neoplasm, obstructive jaundice due to a stone causes scarring of the gallbladder, precluding its distension.
  • Fever can occur in the setting of ascending cholangitis.
  • Hepatomegaly can occur.
  • Rarely, patients present with features of acute pancreatitis or migratory thrombophlebitis.
  • Palpable fixed epigastric masses or supraclavicular nodes are signs of advanced disease and inoperability.

Causes

  • The etiology of the disease is poorly understood.
    • Patients with familial adenomatous polyposis (FAP) have an increased risk of both benign and malignant ampullary tumors.1
    • As many as 50-90% of patients with FAP develop duodenal adenomas, predominantly concentrated on or around the major papilla.2
    • Genomic anomalies may be a factor.3
    • K-ras mutations may be a factor.4

More on Carcinoma of the Ampulla of Vater

Overview: Carcinoma of the Ampulla of Vater
Differential Diagnoses & Workup: Carcinoma of the Ampulla of Vater
Treatment & Medication: Carcinoma of the Ampulla of Vater
Follow-up: Carcinoma of the Ampulla of Vater
Multimedia: Carcinoma of the Ampulla of Vater
References
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References

  1. Burke CA, Beck GJ, Church JM, et al. The natural history of untreated duodenal and ampullary adenomas in patients with familial adenomatous polyposis followed in an endoscopic surveillance program. Gastrointest Endosc. Mar 1999;49(3 Pt 1):358-64. [Medline].

  2. Griffioen G, Bus PJ, Vasen HF, et al. Extracolonic manifestations of familial adenomatous polyposis: desmoid tumours, and upper gastrointestinal adenomas and carcinomas. Scand J Gastroenterol Suppl. 1998;225:85-91. [Medline].

  3. Scarpa A, Zamboni G. Genomic anomalies in pancreatic tumors other than common adenocarcinoma. Ann N Y Acad Sci. Jun 30 1999;880:179-90. [Medline].

  4. Berndt C, Haubold K, Wenger F, et al. K-ras mutations in stools and tissue samples from patients with malignant and nonmalignant pancreatic diseases. Clin Chem. Oct 1998;44(10):2103-7. [Medline].

  5. Menzel J, Hoepffner N, Sulkowski U, et al. Polypoid tumors of the major duodenal papilla: preoperative staging with intraductal US, EUS, and CT--a prospective, histopathologically controlled study. Gastrointest Endosc. Mar 1999;49(3 Pt 1):349-57. [Medline].

  6. John TG, Greig JD, Carter DC, Garden OJ. Carcinoma of the pancreatic head and periampullary region. Tumor staging with laparoscopy and laparoscopic ultrasonography. Ann Surg. Feb 1995;221(2):156-64. [Medline].

  7. Willett CG, Warshaw AL, Convery K, et al. Patterns of failure after pancreaticoduodenectomy for ampullary carcinoma. Surg Gynecol Obstet. Jan 1993;176(1):33-8. [Medline].

  8. Barton RM, Copeland EM 3rd. Carcinoma of the ampulla of Vater. Surg Gynecol Obstet. Mar 1983;156(3):297-301. [Medline].

  9. Yeung RS, Weese JL, Hoffman JP, et al. Neoadjuvant chemoradiation in pancreatic and duodenal carcinoma. A Phase II Study. Cancer. Oct 1 1993;72(7):2124-33. [Medline].

  10. Wagle PK, Joshi RM, Mathur SK. Pancreaticoduodenectomy for periampullary carcinoma. Indian J Gastroenterol. Mar-Apr 2001;20(2):53-5. [Medline].

  11. Toh SK, Davies N, Dolan P, et al. Good outcome from surgery for ampullary tumour. Aust N Z J Surg. Mar 1999;69(3):195-8. [Medline].

  12. Sohn TA, Lillemoe KD, Cameron JL, et al. Reexploration for periampullary carcinoma: resectability, perioperative results, pathology, and long-term outcome. Ann Surg. Mar 1999;229(3):393-400. [Medline].

  13. AJCC Cancer Staging Manual. Exocrine pancreas. In: American Joint Committee on Cancer Manual. 5th ed. Philadelphia, Pa: Lippincott-Raven;1997:121-6.

  14. Conlon KC. Carcinoma of the ampulla of vater: a distinct disease entity?. Ann Surg Oncol. Dec 2003;10(10):1136-7. [Medline].

  15. el-Ghazzawy AG, Wade TP, Virgo KS, et al. Recent experience with cancer of the ampulla of Vater in a national hospital group. Am Surg. Jul 1995;61(7):607-11. [Medline].

  16. Gastrointestinal Tumor Study Group. Further evidence of effective adjuvant combined radiation and chemotherapy following curative resection of pancreatic cancer. Cancer. Jun 15 1987;59(12):2006-10. [Medline].

  17. Hartenfels IM, Dukat A, Burg J, Hansen M, Jung M. [Adenomas of Vater's ampulla and of the duodenum. Presentation of diagnosis and therapy by endoscopic interventional and surgical methods]. Chirurg. Mar 2002;73(3):235-40. [Medline].

  18. Kennedy EP, Yeo CJ. Pancreaticoduodenectomy with extended retroperitoneal lymphadenectomy for periampullary adenocarcinoma. Surg Oncol Clin N Am. Jan 2007;16(1):157-76. [Medline].

  19. Martin ED. Anatomopathologie des tumeurs oddiennes. In: Les tumeurs oddiennes. 1978:35-52.

  20. Paraskevas KI, Avgerinos C, Manes C, Lytras D, Dervenis C. Delayed gastric emptying is associated with pylorus-preserving but not classical Whipple pancreaticoduodenectomy: a review of the literature and critical reappraisal of the implicated pathomechanism. World J Gastroenterol. Oct 7 2006;12(37):5951-8. [Medline].

  21. Park JS, Yoon DS, Kim KS, Choi JS, Lee WJ, Chi HS. Factors influencing recurrence after curative resection for ampulla of Vater carcinoma. J Surg Oncol. Mar 15 2007;95(4):286-90. [Medline].

  22. Rosen M, Zuccaro G, Brody F. Laparoscopic resection of a periampullary villous adenoma. Surg Endosc. Aug 2003;17(8):1322-3. [Medline].

  23. Sarmiento JM, Nagomey DM, Sarr MG, Farnell MB. Periampullary cancers: are there differences?. Surg Clin North Am. Jun 2001;81(3):543-55. [Medline].

  24. Sarr MG, Cameron JL. Surgical palliation of unresectable carcinoma of the pancreas. World J Surg. Dec 1984;8(6):906-18. [Medline].

  25. Todoroki T, Koike N, Morishita Y, et al. Patterns and predictors of failure after curative resections of carcinoma of the ampulla of Vater. Ann Surg Oncol. Dec 2003;10(10):1176-83. [Medline].

  26. Van Heek NT, De Castro SM, van Eijck CH, et al. The need for a prophylactic gastrojejunostomy for unresectable periampullary cancer: a prospective randomized multicenter trial with special focus on assessment of quality of life. Ann Surg. Dec 2003;238(6):894-902; discussion 902-5. [Medline].

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Further Reading

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Keywords

periampullary carcinoma, periampullary malignancy, ampullary carcinoma, ampullary cancer, ampullary cancer treatment, ampullary cancer diagnosis, ampullary cancer symptoms, ampullary cancer pictures, carcinoma of papilla of Vater, adenocarcinomas, neuroendocrine tumors, cystadenomas, adenomas, adenocarcinoma of the ampulla of Vater, Courvoisier sign, familial adenomatous polyposis, FAP, duodenal adenomas, endoscopic ultrasonography, EUS, endoscopic retrograde cholangiopancreatography, ERCP, percutaneous transhepatic cholangiography, PTC, kocherization, pancreaticoduodenectomy, pylorus-preserving pancreaticoduodenectomy, transduodenal excision

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Contributor Information and Disclosures

Author

Nafisa K Kuwajerwala, MD, Staff Surgeon, Breast Oncology, William Beaumont Hospital
Nafisa K Kuwajerwala, MD is a member of the following medical societies: American College of Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Pankaj Chaturvedi, MBBS, MS, Associate Professor, Head and Neck Surgery, Department of Surgical Oncology, Tata Memorial Hospital, India
Pankaj Chaturvedi, MBBS, MS is a member of the following medical societies: American Association for the Advancement of Science and Association of Surgeons of India
Disclosure: Nothing to disclose.

Ronald S Chamberlain, MD, Chairman, Chief, Department of Surgery, Saint Barnabas Medical Center
Ronald S Chamberlain, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Study of Liver Diseases, American College of Surgeons, American Medical Association, Phi Beta Kappa, Society for Surgery of the Alimentary Tract, and Society of Surgical Oncology
Disclosure: Nothing to disclose.

Venkata Subramanian Kanthimathinathan, MD, Staff Physician, Department of General Surgery, Loma Linda University Medical Center
Disclosure: Nothing to disclose.

Uma Chaturvedi, MD, MBBS, DPB, Lecturer, Department of Pathology, KJ Somaiya Hospital and Research Center, India
Disclosure: Nothing to disclose.

Gunateet Goswami, MD, Consulting Staff, Internal Medicine Associates, Mount Clemens, Michigan; Consulting Staff, Department of Cardiology, Henry Ford Hospital
Gunateet Goswami, MD is a member of the following medical societies: American Medical Association, American Society of Echocardiography, and Michigan State Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Michael Perry, MD, MS, MACP, Nellie B Smith Chair of Oncology Emeritus, Professor, Department of Internal Medicine, Division of Hematology and Oncology, University of Missouri /Ellis Fischel Cancer Center
Michael Perry, MD, MS, MACP is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Association, American Society of Clinical Oncology, American Society of Hematology, International Association for the Study of Lung Cancer, and Missouri State Medical Association
Disclosure: Bionumerik Consulting fee Consulting; Proactya Consulting fee Consulting; GSK Consulting fee Consulting; NovoNordisk Consulting fee Consulting; Amgen Honoraria Speaking and teaching; GSK Consulting fee Speaking and teaching

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Benjamin Movsas, MD, Vice-Chairman, Department of Radiation Oncology, Fox Chase Cancer Center
Benjamin Movsas, MD is a member of the following medical societies: American College of Radiology, American Radium Society, and American Society for Therapeutic Radiology and Oncology
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Jules E Harris, MD, Clinical Professor of Medicine, Division of Hematology/Medical Oncology, Department of Internal Medicine, University of Arizona College of Medicine at Tucson; Consulting Staff, Arizona Cancer Center
Jules E Harris, MD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Immunologists, American Society of Hematology, and Central Society for Clinical Research
Disclosure: GlobeImmune Salary Consulting; Amplimed Consulting fee Consulting

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