Carcinoma of the Ampulla of Vater 

  • Author: Nafisa K Kuwajerwala, MD; Chief Editor: Jules E Harris, MD   more...
 
Updated: Oct 27, 2011
 

Background

Carcinoma of the ampulla of Vater, shown in the image below, is a rare 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.

Endoscopic view of an ampullary carcinoma. Endoscopic view of an ampullary carcinoma.

The common bile duct merges with the pancreatic duct of Wirsung to form a common channel that 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.

Carcinoma of the ampulla of Vater tends to manifest early due to biliary outflow obstruction, as opposed to pancreatic neoplasms that often are advanced at the time of diagnosis.

Surgical resection with curative intent is the only option for long-term survival. Surgical, endoscopic, 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.

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Pathophysiology

Periampullary carcinoma includes tumors arising in the head, neck, or uncinate process of the pancreas, tumors arising in the distal common bile duct, tumors arising in the duodenum, as well as tumors arising from the ampulla of Vater.

Review of the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute indicates adenocarcinoma is the most frequently identified histology for ampullary cancer. Adenocarcinoma (not otherwise specified [NOS]) was reported in 65% of cases. Carcinoma (NOS) was reported in 8.1%; adenocarcinoma arising from adenoma (adenocarcinoma in villous adenoma, in tubulovillous adenoma, in adenomatous polyp and villous adenocarcinoma) was third most common in 7.5%. Other pathologic diagnoses reported included papillary adenocarcinoma (5.6%), mucinous adenocarcinoma (4.7%), and signet ring cell carcinoma (2%).[1]

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.

The concept of ampullary carcinoma as a distinct entity is challenged by the categorization of tumors into intestinal type and biliopancreatic type histologically. A review of 118 adenocarcinomas revealed that the biliopancreatic type had a worse prognosis while the intestinal type may behave more like duodenal carcinoma.[2]

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Epidemiology

Frequency

United States

Carcinoma of the ampulla of Vater is an uncommon tumor. Between 1973 and 2005, 5,625 cases were recorded in the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute. Ampullary cancer now accounts for approximately 0.5% of all gastrointestinal tract malignancies.[1] The incidence has been increasing since 1973 at an annual percentage rate of 0.9%.[1] Prior to the recently reported increase, the quoted incidence for ampullary carcinoma was 0.2% of all gastrointestinal malignancies and 6% of all periampullary tumors.[3]

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 approximately 40%[4, 5, 6] to 67% at best.[3]

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, intra-abdominal sepsis, thrombophlebitis, and marginal ulceration are the most common complications.

Postoperative mortality rates in the best centers are 1-2%.

Race

Ampullary carcinoma may be more common in Caucasians than in African Americans.[1]

Sex

A higher rate of ampullary cancer is observed in men.[1]

Age

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

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Contributor Information and Disclosures
Author

Nafisa K Kuwajerwala, MD  Staff Surgeon, Breast Care Center, William Beaumont Hospital

Nafisa K Kuwajerwala, MD is a member of the following medical societies: American College of Surgeons, American Society of Breast Disease, and American Society of Breast 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, American Head and Neck Society, Association of Surgeons of India, and Indian Academy of Tropical Parasitology

Disclosure: Nothing to disclose.

Ronald S Chamberlain, MD  Chairman, Surgeon-in-Chief, Department of Surgery, Director, Gastrointestinal Care Center, Medical Student Clerkship Director, Medical Executive Committee Member, St Barnabas Medical Center; Associate Professor of Surgery, New York College of Osteopathic Medicine; Associate Professor of Surgery, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

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: Wyeth Honoraria Other; Ethicon Honoraria Speaking and teaching; Sanofi Aventis Honoraria Other

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.

Julie A Stein, MD  Clinical Faculty, Hepatobiliary and Pancreatic Surgery, Department of Surgery, William Beaumont Hospital

Julie A Stein, MD is a member of the following medical societies: American College of Surgeons, American College of Surgeons Oncology Group, American Hepato-Pancreato-Biliary Association, Pancreas Club, Society for Surgery of the Alimentary Tract, and Society of American Gastrointestinal and Endoscopic Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael Perry, MD, MS, MACP  Nellie B Smith Chair of Oncology Emeritus, Director, Division of Hematology and Medical Oncology, Deputy Director, Ellis Fischel Cancer Center, University of Missouri-Columbia School of Medicine

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: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, 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; 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

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Endoscopic view of an ampullary carcinoma.
Kocherization of the duodenum. For ampullary malignancies greater than 1 cm in size, pancreaticoduodenectomy is the preferred operation. This figure demonstrates the process of kocherization of the duodenum. The second and third portions of the duodenum are mobilized en bloc with the periduodenal nodal tissue. The authors prefer to expose the inferior vena cava (IVC) and remove alveolar tissue, which lies above the IVC en bloc with the specimen.
Periampullary malignancy. Transected pancreas with head. Pancreaticoduodenectomy is the preferred treatment for most periampullary tumors. This picture depicts transection of the pancreas at the pancreatic neck. This particular patient presented with a periampullary malignancy accompanied by jaundice and pancreatitis. A preoperative pancreatic stent (usually unnecessary) is seen within the pancreatic duct.
Carcinoma of the ampulla of Vater. Roux-en-Y reconstruction following completion of a standard pancreaticoduodenectomy.
Double duct sign of periampullary cancers. Note the dilated common bile duct as well as the pancreatic duct. Liver metastatic lesion is also seen.
Distended gall bladder with double duct sign in a patient with periampullary cancer.
 
 
 
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