TUMORS OF THE AMPULLA OF
VATER
Aswad H. Al.Obeidy
FICMS, FICMS GE&Hep
Kirkuk General Hospital
TUMORS OF THE AMPULLA OF VATER
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Has the highest incidence of neoplastic
transformation and malignancy.
Production of local carcinogens through the
combined interactions of the components of bile,
pancreatic juice, and duodenal contents.
Both benign and malignant tumors of the ampulla
of Vater occur.
The benign tumors include adenomas,
gastrointestinal stromal tumors (GISTs) , lipomas,
and neuroendocrine tumors.
Tumors metastatic from other primary sites have
also been reported
ADENOMA AND ADENOCARCINOMA
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Most common malignant tumor of the ampulla of Vater
less than 10% of all periampullary adenocarcinomas
Up to 25% of such tumors that are resectable.
Arise from the mucosal cells of the ampulla of Vater and undergo an
adenoma–carcinoma progression sequence
Benign adenomas may become dysplastic, with subsequent
progression to adenocarcinoma.
80% to 90% of adenocarcinomas were surrounded by benign
adenomas
In many cases evolution from benign to malignant histology was
noted.
Risk factors for ampullary adenocarcinoma include familial
adenomatous polyposis (FAP) and Peutz-Jeghers syndrome. FAP
have higher frequency of ampullary adenomas, 50% to 86%
Clinical Features
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The average age of patients with ampullary adenoma is the mid-50s
For ampullary adenocarcinoma is the mid-60s.
Present most commonly with obstructive jaundice, in 80%
Weight loss occurs in 75% of patients
Abdominal pain in 50%
Occult gastrointestinal bleeding is common, in one third of patients
Nonspecific symptoms such as anorexia, dyspepsia, and malaise
Rarely, with pancreatitis secondary to pancreatic duct obstruction
With features of sphincter of Oddi dysfunction
Physical examination include conjunctival or cutaneous icterus and,
less commonly, hepatomegaly, a distended gall-bladder
Positive fecal occult blood test result
Very rarely distinctive silver stools, which result from a combination
of the absence of bilirubin and the presence of blood.
Diagnosis
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The earliest and most common laboratory abnormality is an
increase in the serum alkaline phosphatase level
Followed by hyperbilirubinemia as the tumor obstructs the bile
duct.
No tumor markers have been identified that are either sensitive or
specific
The first imaging modality should be ultrasonography or CT to
determine the level of biliary obstruction.
Dual-contrast helical CT is the most informative and cost-effective
imaging technique
CT can detect a periampullary mass larger than 1cm,the level of
biliary obstruction, the relationship of the mass with surrounding
vascular structures, and the presence or absence of liver
metastases.
Endoscopy to visualize duodenal mucosal abnormalities
ERCP is often the next procedure for patients with a suspected
ampullary malignancy
EUS has been reported to be both reliable and accurate in the
diagnosis and staging
Staging
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Primary Tumor (T Stage)
T1 Tumor limited to ampulla of Vater
T2 Tumor invades duodenal wall
T3 Tumor invades ≤2 cm into pancreas
T4 Tumor invades >2 cm into pancreas and/or adjacent organs
Regional Lymph Nodes (N Stage)
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis
Distant Metastasis (M Stage)
M0 No distant metastasis
M1 Distant metastasis
TNM Stage Grouping
Stage I
T1
N0
M0
Stage II
T2-3 N0
M0
Stage III
T1-3 N1
M0
Stage IVA T4
N0-1 M0
Stage IVB T1-4 N0-1 M1
Management
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Local Excision
The first local surgical resection of an ampullary tumor was
performed by William S. Halsted in 1899.
Currently, local resection of ampullary tumors is reserved for patients
with a benign adenoma or ampullary neuroendocrine tumor and for
highly selected patients with ampullary adenocarcinoma
The options for local treatment are endoscopic snare removal,
endoscopic ablation, and surgical ampullectomy.
Pedunculated tubular adenomas smaller than 1 cm without severe
dysplasia can be managed endoscopically, usually with snare
excision, sphincterotomy, and temporary placement of a pancreatic
duct stent,the risk of incomplete resection and recurrence is as high
as 20% with this method.
Operative resection
For adenomas larger than 1 cm, operative resection is indicated once
metastatic disease is excluded.
Postoperative morbidity after local ampullectomy is low, and
mortality rates lower than 2% have been reported.
Review of the literature
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Beger and colleagues identified 62 patients who had undergone
ampullectomy for benign neoplasms. After a follow-up period
ranging from 1 to 156 months, all were free of disease.
Branum and associates reported that of 19 patients with benign
neoplasms resected with ampullectomy, disease recurred in 5 at
a mean of 35 months (range, 8 to 72 months); two of these
patients also had FAP.
Mayo Clinic suggesting that such patients may be at risk for the
development of adenocarcinoma at the site of recurrence. Fifty
patients with benign villous ampullary tumor were treated with
local excision, and 17 tumors recurred, with actuarial recurrence
rates of 32% at 5 years and 43% at 10 years. Four of the
recurrences (24%) were adenocarcinomas
Whipple's procedure
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Treatment of choice for patients with ampullary adenocarcinoma
Classic pancreaticoduodenectomy, which includes a distal
gastrectomy, and the pylorus-preserving modification
Local lymph node resection is adequate because there are no data
to support an extended lymph node dissection for ampullary
adenocarcinoma.
Many centers reporting operative mortality rates of less than 5%
Morbidity rates associated with this procedure remain high, from
25% to 50%
In 1997, the group at the Johns Hopkins Hospital reported the
largest single-institution experience in the management of
adenocarcinoma of the ampulla of Vater
One hundred twenty patients with adenocarcinoma were treated
over a 28-year period. Resection was performed in 106 patients
(88%), 105 of whom (99%) underwent either a
pancreaticoduodenectomy (n = 103) or total pancreatectomy (n = 2).
The overall operative mortality rate was 3.8%. One or more
complications occurred in 49 patients, for an overall morbidity rate
of 47%. The most common complication was pancreatic fistula,
which occurred in 23 patients (25%).
Chemotherapy and Radiation Therapy and palliation
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There are no data to support use adjuvant chemoradiation for
ampullary adenocarcinoma.
Limited data demonstrate that adjuvant chemotherapy with 5FU, doxorubicin, and mitomycin C delays tumor recurrence in
patients with ampullary adenocarcinoma
Operative palliation of obstructive jaundice with a Roux-en-Y
hepaticojejunostomy is appropriate.
A gastrojejunostomy also should be performed in patients with
a compromised duodenal lumen to prevent gastric outlet
obstruction before death.
If the tumor is determined preoperatively to be unresectable,
biliary decompression can be ac-complished by means of
endoscopic or percutaneous techniques.
Prognosis
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Survival in patients with unresectable ampullary adenocarcinoma is
5 to 9 months.
The overall 5-year survival rate for patients with resected ampullary
cancer ranges from 25% to 55%
The 5-year survival rate is significantly better than that for patients
with pancreatic adenocarcinoma
In the 1997 Johns Hopkins series, lymph node status, degree of
differentiation, and operative blood loss were significant predictors
of survival
Tumor diameter had no effect on survival, nor did adjuvant therapy
In a series of 101 patients who underwent resection for ampullary
adenocarcinoma at Memorial Sloan-Kettering Cancer Center, the 5year survival rate was 46%, and resection margin status, nodal
status, and tumor differentiation were predictive of survival