January 14, 2007

Ampullary Carcinoma

Synonyms and related 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


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.


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

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.





Bile Duct Strictures
Bile Duct Tumors
Carcinoma of the Ampulla of Vater
Gallbladder Cancer
Lymphoma, Non-Hodgkin
Pancreatic Cancer

Other Problems to be Considered:

Biliary cirrhosis


Lab Studies:

Imaging Studies:

Staging: Over the years, multiple systems for staging this tumor have been proposed.
    • Primary tumor
      • TX – Primary tumor cannot be assessed
      • T0 – No evidence of primary tumor
      • Tis – Carcinoma in situ
      • T1 – Tumor limited to ampulla of Vater
      • T2 – Tumor invades duodenal wall
      • T3 – Tumor invades less than 2 cm into pancreas
      • T4 – Tumor invades more than 2 cm into pancreas or other organs
    • Regional lymph nodes
      • NX – Regional lymph nodes cannot be assessed
      • N0 – No regional lymph node metastases
      • N1 – Lymph node metastases
    • Distant metastases
      • MX – Presence of distant metastases cannot be assessed
      • M0 – No distant metastases
      • M1 – Distant metastases


Surgical Care: The standard surgical approach is pancreaticoduodenal resection (Whipple procedure). The procedure involves en bloc resection of the gastric antrum and duodenum; a segment of the first portion of the jejunum, gallbladder, and distal common bile duct; the head and often the neck of the pancreas; and adjacent regional lymph nodes.

Results after radical resection of ampullary of Vater carcinoma have been improving. During the past decade, 5-year survival rates have ranged from 20-61%, averaging higher than 35%. The reported mortality rates from this operation are decreasing. A summary follows in Table 2.

Table 2. Results of Pancreaticoduodenal Resection for Carcinoma of the Ampulla of Vater

Institution YearPatients, #Resected, # Mortality Rate, %5-Year Survival Rate, %
Cleveland Clinica1950-19845959837
Leicester Royal Infirmary, United Kingdomb1972-198452241356
University of Alabamac1953-198824241361
Mayo Clinicd1965-19891041045.734
Montebelluna Hospital, Italye1971-19903631356
US Veterans Hospitalf1971-1993123641420
Academic Medical Center, Amsterdamg1984-19926762650
Hannover Hospital, Germanyh1971-19938785938
Johns Hopkinsi1969-1996120106438
Memorial Sloan Ketteringj1983-1995123101544
Catholic University, Italyk1981-20029464964
a Tarazi, 1986
b Neoptolemos, 1988
c Shutze, 1990
d Monson, 1991
e Sperti, 1994
f El-Ghazzawy, 1995
g Allema, 1995
h Klempnauer, 1995
i Talamini, 1997
j Howe, 1998
k Di Giorgio, 2005


Further Outpatient Care:

No comments: