Ampullary Carcinoma

Brooke R. Jeffrey, MD

Amir A. Borhani, MD

Key Facts

Terminology

Malignant epithelial neoplasm (adenocarcinoma) arising from ampulla of Vater

Imaging

Ampullary mass with variable attenuation (most often hypodense) distinct from pancreas with dilated CBD and PD; nodal or liver mets in advanced cases
“Double duct” sign with obstruction of common bile duct and pancreatic duct

Top Differential Diagnoses

Pancreatic head carcinoma invading ampulla
Adenoma of ampulla
Distal cholangiocarcinoma
Mesenchymal tumor of ampulla
Duodenal carcinoma (adenocarcinoma)

Pathology

Lobulated soft tissue mass arising from ampulla of Vater
Markedly increased incidence in hereditary polyposis syndromes (e.g., familial adenomatosis coli, hereditary nonpolyposis colon cancer, etc.)

Clinical Issues

Jaundice (80%), weight loss (61%), abdominal pain, & back pain (46%) are most common symptoms
Better prognosis than periampullary carcinoma of biliary or pancreatic origin

Diagnostic Checklist

Duodenal distension with water on CECT key to identifying lesion
Perform dedicated pancreatic protocol when ampullary lesion suspected

(Left) Endoscopic image of the ampulla demonstrates a soft tissue ampullary carcinoma

in this 63-year-old man who presented with painless jaundice. Note the multilobulated contour of the mass. (Right) Coronal volume-rendered CECT in the same patient demonstrates a polypoid ampullary carcinoma

that has a heterogeneous CT attenuation. Note the obstruction of the common bile duct

and pancreatic duct

, known as the “double duct” sign.

(Left) Coronal volume-rendered CECT in a 72-year-old man who presented with a 15-pound weight loss and painless jaundice demonstrates a hypodense ampullary carcinoma infiltrating the duodenum

. (Right) Coronal volume-rendered CECT in the same patient reveals a calcification in the mass

, an unusual finding in the setting of ampullary carcinoma. Note the “double duct” sign, with obstruction of the common bile duct

and the pancreatic duct

TERMINOLOGY

Definitions

Malignant epithelial neoplasm (adenocarcinoma) arising from ampulla of Vater

IMAGING

General Features

Best diagnostic clue
- Soft tissue (ST) mass involving ampulla
- “Double duct” sign with obstruction of both common bile duct (CBD) and pancreatic duct (PD)
- Lesion best visualized on CECT when duodenum distended with water
Location
- Within ampulla of Vater or overlying periampullary duodenal mucosa
Size
- 1-4 cm in diameter; mean 2.7 cm
Morphology
- Often lobulated mass

Radiographic Findings

ERCP
- Visible ampullary mass
- Obstruction of CBD and PD
- Useful for biopsy

Fluoroscopic Findings

Upper gastrointestinal: Filling defect in 2nd part of duodenum in region of ampulla of Vater

CT Findings

CECT
- Ampullary mass with variable attenuation (most often hypodense) distinct from pancreas, with dilated CBD and PD
  - Nodal or liver metastases in advanced cases

MR Findings

T1WI
- Hypointense to pancreas on non-fat-suppressed T1WI
T1WI FS
- Isointense or slightly hypointense to pancreas on fat-suppressed T1WI
T2WI
- Intermediate signal ampullary mass
- Dilated main PD and CBD
T1WI C+
- Enhancing ST mass of lower signal than pancreas
MRCP
- Dilated PD and CBD
- Up to 76% accuracy

Ultrasonographic Findings

Grayscale ultrasound
- Dilated CBD and PD
- Ampullary mass usually not visible
- Liver metastases in advanced cases
Color Doppler
- No flow in dilated hypoechoic CBD and PD
Endoscopic US
- Useful for staging (most accurate modality for T staging) and biopsy
- Detection of nodal metastases

Angiographic Findings

Conventional
- Superselective injection of gastroduodenal artery demonstrates hypovascular mass

Nuclear Medicine Findings

PET
- May demonstrate liver metastases as FDG-avid lesions
Hepatobiliary scintigraphy
- Dilated bile ducts

Imaging Recommendations

Best imaging tool
- CECT with dedicated biphasic pancreatic protocol or multiphasic MR with MRCP
Protocol advice
- Patient to drink 16 oz of water just prior to CT
- Arterial phase acquisition: Rapid bolus injection of 150 mL IV contrast (4-5 mL/s); 1.25 mm collimation after 10-second delay
- Venous phase acquisition at 70 seconds; 5 mm collimation
- Reconstruct pancreas images: 20 cm field of view
- Additional reformations including curved planar reformat of PD and CBD useful