eMedicine Specialties > Oncology > Carcinomas of the Gastrointestinal Tract

Ampullary Carcinoma

Author: Vivek K Mehta, MD, Radiation Oncologist, Director, Center for Advanced Targeted Radiotherapies, Department of Radiation Oncology, Swedish Cancer Institute, Seattle, Washington
Coauthor(s): George Fisher, MD, PhD, Associate Professor, Department of Internal Medicine, Division of Medical Oncology, Stanford University School of Medicine
Contributor Information and Disclosures

Updated: Jul 18, 2006

Introduction

Background

Carcinoma of the ampulla of Vater is defined as a malignant tumor arising in the last centimeter of the common bile duct where it passes through the wall of the duodenum and ampullary papilla. The pancreatic duct (of Wirsung) and common bile duct merge and exit by way of the ampulla into the duodenum. The ductal epithelium in these areas is columnar and resembles that of the lower common bile duct.

Adenocarcinoma of the ampulla of Vater is a relatively uncommon tumor that accounts for approximately 0.2% of gastrointestinal tract malignancies and approximately 7% of all periampullary carcinomas.

Pathophysiology

The periampullary region is anatomically complex, representing the junction of 3 different epithelia, pancreatic ducts, bile ducts, and duodenal mucosa. Carcinomas originating in the ampulla of Vater by gross inspection can arise from 1 of 4 epithelial types, (1) terminal common bile duct, (2) duodenal mucosa, (3) pancreatic duct, or (4) ampulla of Vater.

Distinguishing between true ampullary cancers and periampullary tumors is critical to understanding the biology of these lesions. Each type of mucosa produces a different pattern of mucus secretion. In a complete histochemical study, Dawson et al divided acid mucins into sulphomucins and sialomucins and demonstrated that ampullary tumors secreting sialomucins had a better prognosis (100% vs 27% 5-y survival rate). In general, ampullary cancers produce sialomucins, whereas periampullary tumors secrete sulfated mucins. Other investigators have confirmed the prognostic power of the pattern of mucin secretion.

Immunohistochemical stains for expressions of carcinoembryonic antigen (CEA), carbohydrate antigen (CA) 19-9, Ki-67, and p53 have been studied for prognostic power. In a series of 45 patients, expression of CA 19-9 labeling intensity and apical localization both were statistically significant predictors of poor prognosis. The 5-year survival rates were markedly different between tumors that expressed CA 19-9 and those that did not (36% vs 100%). CEA expression also might be a marker for prognosis, but it is much weaker. Ki-67 and p53 were not demonstrated to have an effect on outcome. Research along these avenues ultimately might provide the rationale for discriminative administration of adjuvant therapy.

Frequency

United States

Adenocarcinoma of the ampulla of Vater is a relatively uncommon tumor that accounts for approximately 0.2% of gastrointestinal tract malignancies and approximately 7% of all periampullary carcinomas.

Mortality/Morbidity

Pancreaticoduodenectomy is a formidable operation, and the morbidity and mortality rates associated with this procedure historically have been high.

  • Until recently, the operative mortality rate was reported to be approximately 20%. In the past few years, several centers have reported large series with an operative mortality rate in the range of 5%. A recent review of the last 130 pancreaticoduodenectomies performed at Stanford University Medical Center over the last 5 years reveals an operative mortality rate of 3%. This improvement can be attributed to increased surgical experience, improved patient selection, improved anesthesia, better preoperative imaging, and general improvement in the management of ill patients.
  • The morbidity rate associated with the surgery is approximately 65%. In some series, 13% of patients required a repeat laparotomy for complications. Patients may experience fistula formation, delayed intestinal function, pneumonitis, intra-abdominal infection, abscess, or thrombophlebitis. Marginal ulceration, diabetes, pancreatic dysfunction (steatorrhea), and gastrointestinal motility disorder all can manifest as late complications of the surgery.

Race

Because carcinoma of ampulla of Vater is relatively uncommon, studies of the patterns of occurrence among different ethnic groups have not been conducted.

Sex

In most published series, the incidence of carcinoma of the ampulla of Vater is relatively equal between men and women. The rarity of this tumor precludes a careful and accurate estimate of the true incidence between the sexes.

Clinical

History

  • Patients with carcinoma of the ampulla of Vater often complain of anorexia, nausea, vomiting, jaundice, pruritus, or weight loss.
  • Many patients complain of abdominal pain.
  • Diarrhea, a common but not universal symptom, might be associated with an absence of lipase within the gut because of pancreatic duct obstruction.

Physical

  • Upon physical examination, some patients might demonstrate a distended, palpable Courvoisier gallbladder (ie, palpable gall bladder in a patient with jaundice).
  • Fever can be present, particularly when the biliary tract has been explored previously (eg, after common duct exploration for stones).
  • A rising bilirubin level due to obstructive jaundice often is the sole presenting symptom.
  • Ultrasound of the abdomen is the initial study to evaluate the common bile duct or pancreatic ducts (dilation of these ducts essentially is diagnostic for extrahepatic obstruction). However, 10-15% of patients with normal common bile duct findings after ultrasound still might have extrahepatic biliary obstruction on computed tomography (CT) scan findings. Biliary or pancreatic ductal dilation can explain abdominal pain, even with localized and noninvasive disease.
  • CT scan often demonstrates a mass but is not helpful in differentiating ampullary carcinoma from tumors of the head of the pancreas or periampullary region. If the lesion is smaller than 2 cm, pancreatic or bile duct dilation might be the only abnormalities noted on CT scan findings.
  • Such findings are highly suggestive of pancreatic malignancy and require further evaluation, usually with endoscopic retrograde cholangiopancreatography (ERCP). Findings on ERCP that suggest pancreatic cancer include irregular pancreatic duct narrowing, displacement of the main pancreatic duct, destruction or displacement of the side branches of the duct, and pooling of contrast material in necrotic areas of tumor. Both CT scan and ultrasound findings can help reveal metastatic disease in the liver or regional lymph nodes.
  • Dynamic CT scanning, ie, high-speed scans obtained during rapid intravenous administration of iodinated contrast material, can reveal tumor involvement of the vasculature. Some centers still rely on angiography to help identify patients with potentially resectable disease.

More on Ampullary Carcinoma

Overview: Ampullary Carcinoma
Differential Diagnoses & Workup: Ampullary Carcinoma
Treatment & Medication: Ampullary Carcinoma
Follow-up: Ampullary Carcinoma
References
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References

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Further Reading

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Keywords

cancer, carcinoma, bile duct cancer, common bile duct, duodenal mucosa, pancreatic duct, adenocarcinoma, gastrointestinal malignancy, gastrointestinal cancer, GI cancer, GI malignancy, ampulla of Vater, ampullary carcinoma, periampullary carcinoma, cancer of the ampulla of Vater, pancreaticoduodenal resection, Whipple procedure

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Contributor Information and Disclosures

Author

Vivek K Mehta, MD, Radiation Oncologist, Director, Center for Advanced Targeted Radiotherapies, Department of Radiation Oncology, Swedish Cancer Institute, Seattle, Washington
Vivek K Mehta, MD is a member of the following medical societies: American Society for Therapeutic Radiology and Oncology, Phi Beta Kappa, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

George Fisher, MD, PhD, Associate Professor, Department of Internal Medicine, Division of Medical Oncology, Stanford University School of Medicine
George Fisher, MD, PhD is a member of the following medical societies: American Cancer Society, American Medical Association, and American Society of Clinical Oncology
Disclosure: Nothing to disclose.

Medical Editor

Clarence Sarkodee-Adoo, MD, Consulting Staff, Department of Bone Marrow Transplantation, City of Hope Samaritan BMT Program
Clarence Sarkodee-Adoo, MD is a member of the following medical societies: American Society of Clinical Oncology
Disclosure: Nothing to disclose.

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, American Society for Therapeutic Radiology and Oncology, and American Society of Clinical 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

John S Macdonald, MD, Professor of Medicine, New York Medical College; Chief, Division of Medical Oncology, St Vincent's Hospital and Medical Center; Medical Director, Saint Vincent's Comprehensive Cancer Center
Disclosure: Nothing to disclose.

 
 
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