Ampullary adenocarcinoma
Updates to Article Attributes
Ampullary adenocarcinomas are rare biliary tumours arising from the distal biliary epithelium at the ampulla of Vater.
Although classically presenting on imaging with the double duct sign, the tumour itself may be occult or of limited characterisation on imaging.
Epidemiology
These are rare tumours, with an adulthood estimated incidence of 0.70 cases out of.7 in 100.000,000 men and 0.45 cases out ofin 100.000,000 women in the United States 2.
There is an association with Gardner syndrome.
Clinical presentation
These patients typically present with obstructive jaundice and abdominal pain, but the presentation may be vague with other nonspecificnon-specific symptoms.
Radiographic features
USUltrasound, CT, and MRI allmay demonstrate the double duct sign: simultaneous (simultaneous dilatation of the common bile and pancreatic ducts;) which is seen in ~52% of cases 1.
CT
may demonstrate a small, solid, enhancing
smalltumour in the ampullary region protruding into the second portion of the duodenum 1may show lobulated or infiltrative margins
have been described1discrete tumours may not be visible 1,3
CT
IV Cholangiogramcholangiogramitis usually not performed given the presence of biliary obstruction and raised bilirubin levels
MRI
MRCP has been described as more sensitive than other imaging modalities for depicting ampullary lesions, but withhas low specificity though 3.
-
T2
-
itmay delineate the presence of an ampullary mass or papillary bulging
-
besides thein addition to biliary tree dilatation, may show irregular narrowing of the distal common bile duct
-
DWI/ADC:
somethere may be a degree of restricted diffusionhelp in differentiatingwhich helps to differentiate it from benign lesions 3
Treatment and prognosis
Tumours that are occult on imaging are also frequently occult at ERCP/endoscopy, with the diagnosis only achieved after papillotomy/biopsy 1.
Differential diagnosis
papillitis
papillary stenosis (e.g. post-inflammatory)
-<p><strong>Ampullary adenocarcinomas</strong> are rare biliary tumours arising from the distal biliary epithelium at the <a href="/articles/ampulla-of-vater-1">ampulla of Vater</a>. </p><p>Although classically presenting on imaging with the double duct sign, the tumour itself may be occult or of limited characterisation imaging. </p><h4>Epidemiology</h4><p>These are rare tumours, with an adulthood estimated incidence of 0.70 cases out of 100.000 men and 0.45 cases out of 100.000 women in the United States <sup>2</sup>. </p><h4>Clinical presentation </h4><p>These patients typically present with obstructive jaundice and abdominal pain, but presentation may be vague with other nonspecific symptoms. </p><h4>Radiographic features </h4><p>US, CT, and MRI all demonstrate the <a href="/articles/double-duct-sign">double duct sign</a>: simultaneous dilatation of the common bile and pancreatic ducts; seen in ~52% of cases <sup>1</sup>. </p><h5>CT</h5><ul>-<li>may demonstrate a solid enhancing small tumour in the ampullary region protruding into the second portion of the duodenum <sup>1</sup>-</li>-<li>lobulated or infiltrative margins have been described <sup>1</sup>-</li>-<li>discrete tumours may not be visible <sup>1,3</sup>-</li>- +<p><strong>Ampullary adenocarcinomas</strong> are rare biliary tumours arising from the distal biliary epithelium at the <a href="/articles/ampulla-of-vater-1">ampulla of Vater</a>. </p><p>Although classically presenting on imaging with the <a href="/articles/double-duct-sign" title="Double duct sign">double duct sign</a>, the tumour itself may be occult or of limited characterisation on imaging. </p><h4>Epidemiology</h4><p>These are rare tumours, with an adulthood estimated incidence of 0.7 in 100,000 men and 0.45 in 100,000 women in the United States <sup>2</sup>.</p><p>There is an association with <a href="/articles/gardner-syndrome" title="Gardner syndrome">Gardner syndrome</a>.</p><h4>Clinical presentation </h4><p>These patients typically present with obstructive jaundice and abdominal pain, but the presentation may be vague with other non-specific symptoms. </p><h4>Radiographic features </h4><p>Ultrasound, CT, and MRI may demonstrate the <a href="/articles/double-duct-sign">double duct sign</a> (simultaneous dilatation of the common bile and pancreatic ducts) which is seen in ~52% of cases <sup>1</sup>.</p><h5>CT</h5><ul>
- +<li><p>may demonstrate a small, solid, enhancing tumour in the ampullary region protruding into the second portion of the duodenum <sup>1</sup></p></li>
- +<li><p>may show lobulated or infiltrative margins <sup>1</sup></p></li>
- +<li><p>discrete tumours may not be visible <sup>1,3</sup></p></li>
- +<li><p><a href="/articles/ct-cholangiography-protocol">CT cholangiogram</a> is usually not performed given the presence of biliary obstruction and raised bilirubin levels</p></li>
- +</ul><h5>MRI</h5><p>MRCP has been described as more sensitive than other imaging modalities for depicting ampullary lesions, but has low specificity <sup>3</sup>. </p><ul>
-<a href="/articles/ct-cholangiography-protocol">CT IV Cholangiogram</a> it is usually not performed given biliary obstruction and raised bilirubin levels</li>-</ul><h5>MRI</h5><p>MRCP has been described as more sensitive than other imaging modalities for depicting ampullary lesions, but with low specificity though <sup>3</sup>. </p><ul>-<li>-<strong>T2</strong><ul>-<li>it may delineate the presence of an ampullary mass or papillary bulging</li>-<li>besides the biliary tree dilatation, may show irregular narrowing of the distal common bile duct</li>- +<p><strong>T2</strong></p>
- +<ul>
- +<li><p>may delineate the presence of an ampullary mass or papillary bulging</p></li>
- +<li><p>in addition to biliary tree dilatation, may show irregular narrowing of the distal common bile duct</p></li>
-<li>-<strong>DWI/ADC:</strong> some degree of restricted diffusion help in differentiating from benign lesions <sup>3</sup>-</li>-</ul><h4>Treatment and prognosis</h4><p>Tumours that are occult on imaging are also frequently occult at ERCP/endoscopy, with the diagnosis only achieved after papillotomy/biopsy <sup>1</sup>.</p><h4>Differential diagnosis</h4><ul>-<li><a href="/articles/ampullary-adenoma">ampullary adenoma</a></li>-<li>papillitis</li>-<li>papillary stenosis (e.g. post-inflammatory) </li>- +<li><p><strong>DWI/ADC:</strong> there may be a degree of restricted diffusion which helps to differentiate it from benign lesions <sup>3</sup></p></li>
- +</ul><h4>Treatment and prognosis</h4><p>Tumours that are occult on imaging are also frequently occult at <a href="/articles/endoscopic-retrograde-cholangiopancreatography" title="ERCP">ERCP</a>/endoscopy, with the diagnosis only achieved after papillotomy/biopsy <sup>1</sup>.</p><h4>Differential diagnosis</h4><ul>
- +<li><p><a href="/articles/ampullary-adenoma">ampullary adenoma</a></p></li>
- +<li><p>papillitis</p></li>
- +<li><p>papillary stenosis (e.g. post-inflammatory) </p></li>
Image 1 Pathology (H&E) ( update )
Image 2 Fluoroscopy (ERCP) ( update )
Image 3 CT (Coronal) ( update )
Image 8 CT (C+ delayed) ( update )
Image 9 CT (C+ portal venous phase) ( update )
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