Ampullary Carcinoma

Published on 18/07/2015 by admin

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Last modified 22/04/2025

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 Well defined/lobulated or poorly marginated

image Almost always obstructs common bile duct (CBD)
image Pancreatic duct (PD) obstructed in only ∼ 50%, and usually does not cause upstream pancreatic atrophy
image Small masses often not visualized on CT

– Secondary signs should suggest presence of tumor (e.g., double duct sign, etc.)
• MR: Low signal on T1WI, intermediate signal on T2WI, and hypoenhancing on T1WI C+

image Diffusion-weighted images may increase sensitivity for small masses

TOP DIFFERENTIAL DIAGNOSES

• Pancreatic head adenocarcinoma involving ampulla
• Ampullary adenoma
• Distal CBD cholangiocarcinoma
• Periampullary duodenal carcinoma
• Ampullary carcinoid tumor

PATHOLOGY

• Markedly increased incidence in hereditary polyposis syndromes
• Lesions can be divided into 3 forms

image Tumors arising from duodenal epithelium (intestinal type): Prognosis comparable to duodenal carcinoma
image Tumors arising from pancreaticobiliary epithelium of distal CBD/PD (pancreaticobiliary type): Worst prognosis, with outcomes similar to pancreatic adenocarcinoma
image Intraampullary tumors: Best prognosis with early ductal obstruction and consequent early presentation

CLINICAL ISSUES

• Obstructive jaundice (80%) most common symptom
• Same surgical treatment (Whipple procedure) as for all periampullary neoplasms
image
(Left) Graphic shows ampullary carcinoma image causing obstruction of both the common bile duct (CBD) image and the pancreatic duct (PD) image.

image
(Right) ERCP in a patient with a history of familial polyposis shows a small mass image causing obstruction of the distal common duct at the ampulla. Patients with familial polyposis have a substantially increased risk of developing ampullary (and other) carcinomas. Lifelong surveillance is required to detect bowel, stomach, and biliary tumors.
image
(Left) Coronal CECT demonstrates a classic double-duct sign caused by an ampullary carcinoma image, with obstruction of both the common bile duct image and pancreatic duct image. Note the abrupt occlusion of both ducts by the mass.

image
(Right) Coronal CECT demonstrates a lobulated mass image centered at the ampulla obstructing the pancreatic and common bile ducts. While this was a pancreaticobiliary type lesion at histology, individual subtypes of ampullary carcinoma cannot be distinguished on imaging.

TERMINOLOGY

Definitions

• Heterogeneous group of malignant epithelial neoplasms (adenocarcinoma) arising from ampulla of Vater

IMAGING

General Features

• Best diagnostic clue

image Soft tissue mass centered in ampulla
image Double duct sign with obstruction of both common bile duct (CBD) and pancreatic duct (PD)
• Location

image Centered in region of ampulla of Vater or overlying periampullary duodenal mucosa
• Size

image 1-4 cm in diameter; mean 2.7 cm
• Morphology

image Can be either well-defined lobulated mass or more infiltrative and ill defined

CT Findings

• Mass with variable attenuation (most often hypodense) centered in ampulla

image Can be well defined/lobulated or poorly marginated/infiltrative
image Almost always obstructs CBD with abrupt cut-off of CBD by mass
image PD obstructed in only ∼ 50% of cases, with ampullary tumors much less likely than pancreatic cancer to cause upstream pancreatic atrophy
• Small masses may not be visualized on CT (or any other radiological imaging modality)

image Secondary signs suggest presence of tumor (e.g., double-duct sign, irregular obstructed duct, etc.)
• Most common sites of metastases: Lymph nodes or liver

MR Findings

• Typically low signal on T1WI, intermediate to high signal on T2WI, and hypoenhancing relative to pancreas on T1WI C+
• CBD ± PD dilatation with abrupt ductal cutoff

image MRCP: Abrupt, irregular cut-off of distal CBD
• Diffusion-weighted images may increase sensitivity for small masses (malignant ampullary tumors have lower ADC values than benign lesions)

Ultrasonographic Findings

• Grayscale ultrasound

image May be useful as initial screening tool for detection of biliary dilation in patients presenting with jaundice
image Distal CBD and PD can be difficult to visualize as a result of bowel gas

– Ampullary mass almost never visible on US
image Liver metastases in advanced cases
• Endoscopic US

image Tumors usually visible endoscopically, but endoscopic US may help guide biopsy and improve biopsy yields
image Best modality for T staging (accuracy up to 90%)
image May help detect and biopsy nodal metastases

Radiographic Findings

• ERCP

image Ampullary mass should be readily visible under endoscopy in most cases and can be easily biopsied

– Superior to CT in detecting small tumors
image Cholangiography demonstrates obstruction of CBD and PD with abrupt cut-off at mass

Fluoroscopic Findings

• Upper GI series: Filling defect in 2nd portion of duodenum in region of ampulla of Vater

image No specific features to differentiate ampullary tumor from pancreatic or duodenal malignancy

Angiographic Findings

• Conventional

image Superselective injection of gastroduodenal artery demonstrates hypovascular mass

Nuclear Medicine Findings

• PET

image May demonstrate liver metastases as FDG-avid lesions
• Hepatobiliary scintigraphy

image Dilated bile ducts

Imaging Recommendations

• Best imaging tool

image CECT with dedicated biphasic pancreatic protocol or MR with MRCP
• Protocol advice

image Patients drink 500 cc water immediately prior to CT to distend duodenum (improving visualization of ampulla)
image Dual-phase acquisition (arterial and venous phases) critical for improving visualization of tumor

– Lesions may be more or less conspicuous on each phase
image Coronal multiplanar reformats (MPR) and curved planar reformats helpful for assessing distal CBD and differentiating malignant from benign obstruction

DIFFERENTIAL DIAGNOSIS

Pancreatic Head Adenocarcinoma Involving Ampulla

• Hypoattenuating, infiltrative mass with obstruction of both CBD and PD and involvement of retroperitoneal vasculature
• Frequent upstream pancreatic atrophy (less common with ampullary carcinoma)

Ampullary Adenoma

• Benign lesion which may be precursor to ampullary carcinoma
• Indistinguishable from carcinoma on CT, with slightly lower incidence of severe ductal obstruction
• Double duct sign with dilated CBD and PD
• Variable in size, from 1-5 cm

Distal CBD Cholangiocarcinoma

• 20% of all cholangiocarcinomas occur in distal 1/3 of CBD
• May demonstrate some hypervascularity on arterial phase and delayed enhancement on CECT/MR
• Usually asymmetric wall thickening/enhancement of CBD, but can present as discrete mass
• Biliary obstruction with abrupt cut-off at level of mass
• Worse prognosis than ampullary adenocarcinoma

Periampullary Duodenal Carcinoma

• Duodenum most common site for small bowel adenocarcinoma
• Large duodenal carcinomas may invade ampulla or pancreas and result in ductal obstruction, but ductal obstruction (either PD or CBD) is less common than with ampullary tumors
• May present with gastrointestinal bleeding

Mesenchymal Tumor of Ampulla

• May be hypervascular ampullary mass on CT if neurogenic, i.e., schwannoma or carcinoid
• High T2 signal on MR if neurogenic origin

Ampullary Carcinoid Tumor

• Rare tumor that is avidly hypervascular on arterial phase CECT
• Predisposition for early lymph node and distant metastases, even when primary lesion is quite small

PATHOLOGY

General Features

• Etiology

image Proposed adenoma to adenocarcinoma carcinogenesis sequence (similar to colorectal cancer)

– 60% of adenomas contain foci of invasive carcinoma
– Ampullary adenomas cannot be differentiated from invasive carcinoma on imaging
image Significant tumor heterogeneity, as lesions can be divided into 3 forms, which cannot be differentiated on imaging

– Tumors arising from duodenal epithelium of ampulla (intestinal type)

image Large at presentation with early lymph node metastases
image Prognosis comparable to duodenal carcinoma
– Tumors arising from pancreaticobiliary epithelium of distal CBD or pancreatic duct (pancreaticobiliary type)

image Worst prognosis of 3 types, with biologic behavior similar to pancreatic adenocarcinoma
– Intraampullary tumors with combined duodenal and pancreaticobiliary epithelial origin

image Best prognosis because of origin within ampulla, resulting in early ductal obstruction and early presentation with jaundice
image Less likely to demonstrate significant invasive component
• Genetics

image Markedly increased incidence in hereditary polyposis syndromes (e.g., familial adenomatosis coli, hereditary nonpolyposis colon cancer, etc.)
• Better prognosis than periampullary carcinomas of pancreatic and biliary origin

Staging, Grading, & Classification

• TNM staging system related to nodal and distant metastases

image Nodal metastases outside of peripancreatic region considered M1 lesion
image T1: Lesion confined to ampulla
image T2: Tumor invading duodenal wall
image T3: Pancreatic invasion < 2 cm deep
image T4: Pancreatic invasion > 2 cm deep

Gross Pathologic & Surgical Features

• Lobulated ST mass arising from ampulla of Vater

Microscopic Features

• Malignant ductal epithelial cells with varying degrees of differentiation and necrosis
• Intestinal type: Simple and cribriform glands present with pseudostratified oval nuclei with variable nuclear atypia
• Pancreatobiliary type: Similar histology to ductal adenocarcinoma with single layer of round, markedly atypical nuclei, micropapillary areas
• Spectrum of histology: Dysplasia, carcinoma in situ, frank adenocarcinoma

CLINICAL ISSUES

Presentation

• Most common signs/symptoms

image Obstructive jaundice (80%) most common symptom as nearly all patients have CBD obstruction
image Weight loss (61%), abdominal pain, back pain (46%)
• Other signs/symptoms

image GI bleeding with heme-positive stool
image Diarrhea or steatorrhea
image Nausea, dyspepsia
image Fever and chills from cholangitis
• Clinical profile

image ↑ bilirubin and alkaline phosphatase
image May have ↑ CEA or CA19-9 tumor markers

– Neither sensitive nor specific for presence of tumor
– ↑ preoperative tumor markers associated with poor outcome

Demographics

• Age

image Average age at diagnosis is 65 years (earlier in patients with hereditary polyposis syndromes)
• Gender

image M:F = 2:1
• Ethnicity

image No known ethnic predilection
• Epidemiology

image Rare tumor representing 0.2% of GI tract malignancies and 6% of periampullary lesions
image Incidence: 4-6 per 1 million population
image Associated with smoking (30%) and diabetes (17%)

Natural History & Prognosis

• Depends on histologic type of tumor, as well as nodal and distant metastases at presentation
• Prognosis

image Better than periampullary carcinoma of biliary or pancreatic origin
image Slightly worse than periampullary duodenal carcinoma
image Reported 5-year survival of 64-80% in node-negative patients and 17-50% in node-positive cases
image Best odds of survival: Negative surgical margins, negative nodes, well-differentiated tumors

Treatment

• Preoperative treatment of biliary obstruction with ERCP stent placement
• Pancreatoduodenectomy (Whipple procedure, either classic or pylorus-preserving) in patients with good operative risk
• Local resection (ampullectomy) associated with higher risk of local recurrence and worse long-term survival

image Might be an option in patients with noninvasive tumors with low risk of lymph node metastases

DIAGNOSTIC CHECKLIST

Consider

• Differentiation of ampullary carcinomas from other periampullary tumors (e.g., pancreatic or duodenal adenocarcinoma) may be difficult, but surgical treatment (Whipple procedure) is the same

Image Interpretation Pearls

• Adequate distension of duodenum with water on CECT is key to identifying ampullary mass
• Small ampullary tumors can be very difficult to visualize on CECT or MR, and secondary signs (e.g., double duct sign) should suggest presence of occult tumor
image
(Left) Coronal CECT demonstrates a polypoid mass image centered at the ampulla, representing an ampullary carcinoma. A biliary stent image is partially visualized. The coronal plane is usually the best way of visualizing the ampulla and assessing a potential mass.

image
(Right) Coronal CECT demonstrates an ill-defined hypodense ampullary mass image obstructing the common bile duct image, with only mild dilatation of the pancreatic duct image. This was found to represent an ampullary carcinoma at surgery.
image
(Left) Coronal volume-rendered CECT demonstrates a double-duct sign with obstruction of the common bile duct image and pancreatic duct image by a polypoid ampullary carcinoma image.

image
(Right) Coronal CECT shows a discrete mass image centered around the ampulla with a biliary stent image. Ampullary carcinoma cannot be easily distinguished from a periampullary duodenal carcinoma at imaging, although surgical treatment for both lesions is the same (Whipple procedure).
image
(Left) Coronal CECT demonstrates a rounded, well-defined ampullary mass image obstructing the CBD image. The PD (not shown) was not obstructed. Ampullary carcinomas almost always obstruct the CBD, but obstruct the PD in only 50% of cases.

image
(Right) Axial CECT demonstrates an invasive adenocarcinoma of the ampulla of Vater arising from a villous adenoma. Note the large, circumferential or “apple core” mass image at the junction of the 2nd and 3rd portions of the duodenum.
image
Coronal CECT curved planar reformation of common bile duct shows ampullary carcinoma invading the head of the pancreas. Note the hypodense mass image obstructing the pancreatic and common bile ducts.

image
Coronal CECT minimum-intensity image of ampullary carcinoma invading the head of the pancreas demonstrates a hypodense mass image.
image
Anteroposterior spot film from an upper GI series demonstrates a rounded filling defect from ampullary carcinoma image.
image
Coronal CECT curved planar reformation of the pancreatic duct demonstrates a very small ampullary tumor image obstructing the pancreatic and common bile ducts.
image
Axial CECT thick slab image demonstrates an ampullary carcinoma image with an unusual degree of increased vascularity.
image
Coronal CECT of an ampullary carcinoma demonstrates marked common bile duct obstruction secondary to the mass image.
image
Coronal CECT demonstrates a rounded, well-circumscribed mass image centered in the duodenum at the level of the ampulla, found to represent an ampullary carcinoma at histology. Note the presence of a biliary stent image due to biliary obstruction by the mass.

SELECTED REFERENCES

1. Raman, SP, et al. Abnormalities of the Distal Common Bile Duct and Ampulla: Diagnostic Approach and Differential Diagnosis Using Multiplanar Reformations and 3D Imaging. AJR Am J Roentgenol. 2014; 203(1):17–28.

 

Jang, KM, et al. Added value of diffusion-weighted MR imaging in the diagnosis of ampullary carcinoma. Radiology. 2013; 266(2):491–501.

Westgaard, A, et al. Intestinal-type and pancreatobiliary-type adenocarcinomas: how does ampullary carcinoma differ from other periampullary malignancies? Ann Surg Oncol. 2013; 20(2):430–439.

Chung, YE, et al. Differentiation of benign and malignant ampullary obstructions on MR imaging. Eur J Radiol. 2011; 80(2):198–203.

Chung, YE, et al. Differential features of pancreatobiliary- and intestinal-type ampullary carcinomas at MR imaging. Radiology. 2010; 257(2):384–393.

Barauskas, G, et al. Tumor-related factors and patient’s age influence survival after resection for ampullary adenocarcinoma. J Hepatobiliary Pancreat Surg. 2008; 15(4):423–428.

Carter, JT, et al. Tumors of the ampulla of vater: histopathologic classification and predictors of survival. J Am Coll Surg. 2008; 207(2):210–218.

Chen, WX, et al. Multiple imaging techniques in the diagnosis of ampullary carcinoma. Hepatobiliary Pancreat Dis Int. 2008; 7(6):649–653.

O’Connell, JB, et al. Survival after resection of ampullary carcinoma: a national population-based study. Ann Surg Oncol. 2008; 15(7):1820–1827.

Schirmacher, P, et al. Ampullary adenocarcinoma – differentiation matters. BMC Cancer. 2008; 8:251.

Singh, S, et al. Palliative surgical bypass for unresectable periampullary carcinoma. Hepatobiliary Pancreat Dis Int. 2008; 7(3):308–312.

Smith, RA, et al. Prognosis of resected ampullary adenocarcinoma by preoperative serum CA19-9 levels and platelet-lymphocyte ratio. J Gastrointest Surg. 2008; 12(8):1422–1428.

Ahualli, J. The double duct sign. Radiology. 2007; 244(1):314–315.

Sugita, R, et al. Periampullary tumors: high-spatial-resolution MR imaging and histopathologic findings in ampullary region specimens. Radiology. 2004; 231(3):767–774.

Clements, WM, et al. Ampullary carcinoid tumors: rationale for an aggressive surgical approach. J Gastrointest Surg. 2003; 7(6):773–776.

Duffy, JP, et al. Improved survival for adenocarcinoma of the ampulla of Vater: fifty-five consecutive resections. Arch Surg. 2003; 138(9):941–948. [discussion 948-50].

Handra-Luca, A, et al. Adenomyoma and adenomyomatous hyperplasia of the Vaterian system: clinical, pathological, and new immunohistochemical features of 13 cases. Mod Pathol. 2003; 16(6):530–536.

Lindell, G, et al. Management of cancer of the ampulla of Vater: does local resection play a role? Dig Surg. 2003; 20(6):511–515.

Martin, JA, et al. Ampullary adenoma: clinical manifestations, diagnosis, and treatment. Gastrointest Endosc Clin N Am. 2003; 13(4):649–669.

Nakano, K, et al. Combination therapy of resection and intraoperative radiation for patients with carcinomas of extrahepatic bile duct and ampulla of Vater: prognostic advantage over resection alone? Hepatogastroenterology. 2003; 50(52):928–933.

Smith, TR, et al. Prolapse of the common bile duct with small ampullary villous adenocarcinoma into third part of the duodenum. AJR Am J Roentgenol. 2003; 181(2):599–600.

Trimbath, JD, et al. Attenuated familial adenomatous polyposis presenting as ampullary adenocarcinoma. Gut. 2003; 52(6):903–904.

Irie, H, et al. MR imaging of ampullary carcinomas. J Comput Assist Tomogr. 2002; 26(5):711–717.

Jordan, PH, Jr., et al. Treatment of ampullary villous adenomas that may harbor carcinoma. J Gastrointest Surg. 2002; 6(5):770–775.

Kaiser, A, et al. The adenoma-carcinoma sequence applies to epithelial tumours of the papilla of Vater. Z Gastroenterol. 2002; 40(11):913–920.

Kim, JH, et al. Differential diagnosis of periampullary carcinomas at MR imaging. Radiographics. 2002; 22(6):1335–1352.

Rodriguez, C, et al. How accurate is preoperative diagnosis by endoscopic biopsies in ampullary tumours? Rev Esp Enferm Dig. 2002; 94(10):585–592.

Skordilis, P, et al. Is endosonography an effective method for detection and local staging of the ampullary carcinoma? A prospective study. BMC Surg. 2002; 2(1):1.

Yoshida, T, et al. Hepatectomy for liver metastasis from ampullary cancer after pancreatoduodenectomy. Hepatogastroenterology. 2002; 49(43):247–248.

Eriguchi, N, et al. Carcinoma of the ampulla of Vater associated with other organ malignancies. Kurume Med J. 2001; 48(4):255–259.

Hirata, S, et al. Periampullary choledochoduodenal fistula in ampullary carcinoma. J Hepatobiliary Pancreat Surg. 2001; 8(2):179–181.

Nikfarjam, M, et al. Local resection of ampullary adenocarcinomas of the duodenum. ANZ J Surg. 2001; 71(9):529–533.

Wagle, PK, et al. Pancreaticoduodenectomy for periampullary carcinoma. Indian J Gastroenterol. 2001; 20(2):53–55.

Wittekind, C, et al. Adenoma of the papilla and ampulla—premalignant lesions? Langenbecks Arch Surg. 2001; 386(3):172–175.

Yeo, CJ, et al. Periampullary adenocarcinoma: analysis of 5-year survivors. Ann Surg. 1998; 227(6):821–831.

Talamini, MA, et al. Adenocarcinoma of the ampulla of Vater. A 28-year experience. Ann Surg. 1997; 225(5):590–599. [discussion 599-600].

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