Difference between revisions of "Ampulla of Vater"

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→‎Ampullary adenoma: Added obstruction as a potential mimic of tumor
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The '''ampulla of Vater''', also '''hepatopancreatic ampulla''', is found in the [[duodenum]]. It has a unique histology and is a relatively common site of disease, when duodenal pathology is considered.
The '''ampulla of Vater''', also '''hepatopancreatic ampulla''', is found in the [[duodenum]]. It has a unique histology and is a relatively common site of disease, when duodenal pathology is considered.


=Normal histology=
=Benign=
==Normal histology==
Periampullary:<ref name=pmid23026934/>
Periampullary:<ref name=pmid23026934/>
*Intestinal epithelium with goblet cells.
*Intestinal epithelium with goblet cells.
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*+/-Pancreatic heterotopia - common.
*+/-Pancreatic heterotopia - common.
*+/-Gastric heterotopia - not common.
*+/-Gastric heterotopia - not common.
===Sign out===
<pre>
DUODENAL BULB, BIOPSY:
- GASTRIC HETEROTOPIA.
- NEGATIVE FOR MALIGNANCY.
</pre>
==Acute inflammation==
===Sign out===
<pre>
Ampulla of Vater, Biopsy:
- Small bowel mucosa with foveolar-like glands (in keeping with
  ampulla), reactive epithelial changes and mild acute inflammation.
- NEGATIVE for dysplasia.
</pre>


=Ampullary tumours=
=Ampullary tumours=
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*[[AKA]] ''tumours of the hepatopancreatic ampulla''.  
*[[AKA]] ''tumours of the hepatopancreatic ampulla''.  


==Ampullary Obstruction ==
[[File:5 890331478 sl 1.png|Villous appearing duodenal mass of ampulla, secondary to ampullary duct obstruction]]
[[File:5 890331478 sl 2.png|Villous appearing duodenal mass of ampulla, secondary to ampullary duct obstruction]]
[[File:5 890331478 sl 3.png|Villous appearing duodenal mass of ampulla, secondary to ampullary duct obstruction]]
[[File:5 890331478 sl 4.png|Villous appearing duodenal mass of ampulla, secondary to ampullary duct obstruction]]
[[File:5 890331478 sl 5.png|Villous appearing duodenal mass of ampulla, secondary to ampullary duct obstruction]]<br>
Villous appearing duodenal mass of ampulla, secondary to ampullary duct obstruction. 43 yo White man. A. Plicae show expansion of stroma by edema with inflammation including neutrophils (neutrophils not shown), by foci of lamina propria hemorrhage (red arrows), and by dilated lymphatics (black arrows).  B. A fragment shows dilated ducts on left and center, with connected “proliferating” ducts at right. C. PAS AB stain shows these ducts, by virtue of the thin red line, are ampullary, not Brunner’s glands. D. Nuclei in the proliferated area show striking anisokaryosis and anisonucleosis, as well as hyperchromasia. Note the mitosis (arrow).  This notwithstainging, there is an appearance of connection between the ducts. No net appears. Neither are there half glands or aberrant isolated cells.  E. On PAS AB stain lay a focus of fibrinopurulent exudate near the proliferating area.
==Ampullary adenoma==
==Ampullary adenoma==
===General===
===General===
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*[[Duodenal carcinoma]] secondarily involving the ampulla.
*[[Duodenal carcinoma]] secondarily involving the ampulla.
*[[Pancreatic adenocarcinoma]] secondarily involving the ampulla.
*[[Pancreatic adenocarcinoma]] secondarily involving the ampulla.
====Images====
www:
*[http://ajcp.ascpjournals.org/content/132/4/506/F1.expansion.html Ampullary adenoma (ajcpjournals.org)].<ref name=pmid19762527/>
*[http://ajcp.ascpjournals.org/content/132/4/506/F2.expansion.html Ampullary adenoma - pitfalls (ajcpjournals.org)].<ref name=pmid19762527>{{Cite journal  | last1 = Bellizzi | first1 = AM. | last2 = Kahaleh | first2 = M. | last3 = Stelow | first3 = EB. | title = The assessment of specimens procured by endoscopic ampullectomy. | journal = Am J Clin Pathol | volume = 132 | issue = 4 | pages = 506-13 | month = Oct | year = 2009 | doi = 10.1309/AJCPUZWJ8WA2IHBG | PMID = 19762527 }}</ref>


===Sign out===
===Sign out===
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*Uncommon.  
*Uncommon.  
*Textbook association: [[familial adenomatous polyposis]].<ref name=pmid15756395>{{Cite journal  | last1 = Tran | first1 = TC. | last2 = Vitale | first2 = GC. | title = Ampullary tumors: endoscopic versus operative management. | journal = Surg Innov | volume = 11 | issue = 4 | pages = 255-63 | month = Dec | year = 2004 | doi =  | PMID = 15756395 }}</ref><ref name=pmid9834381>{{Cite journal  | last1 = Soravia | first1 = C. | last2 = Berk | first2 = T. | last3 = Haber | first3 = G. | last4 = Cohen | first4 = Z. | last5 = Gallinger | first5 = S. | title = Management of advanced duodenal polyposis in familial adenomatous polyposis. | journal = J Gastrointest Surg | volume = 1 | issue = 5 | pages = 474-8 | month =  | year =  | doi =  | PMID = 9834381 }}</ref>
*Textbook association: [[familial adenomatous polyposis]].<ref name=pmid15756395>{{Cite journal  | last1 = Tran | first1 = TC. | last2 = Vitale | first2 = GC. | title = Ampullary tumors: endoscopic versus operative management. | journal = Surg Innov | volume = 11 | issue = 4 | pages = 255-63 | month = Dec | year = 2004 | doi =  | PMID = 15756395 }}</ref><ref name=pmid9834381>{{Cite journal  | last1 = Soravia | first1 = C. | last2 = Berk | first2 = T. | last3 = Haber | first3 = G. | last4 = Cohen | first4 = Z. | last5 = Gallinger | first5 = S. | title = Management of advanced duodenal polyposis in familial adenomatous polyposis. | journal = J Gastrointest Surg | volume = 1 | issue = 5 | pages = 474-8 | month =  | year =  | doi =  | PMID = 9834381 }}</ref>
*Prognosis guarded but significantly better than [[pancreatic ductal adenocarcinoma]] - 5 year survival ~40% for ampullary carcinomas vs. 10% for pancreatic adenocarcinoma.<ref name=pmid23026934/>
===Gross===
*Ampullary carcinomas are classified by site.
*Modest differences exist in survival between the sites.


====Classification====
====Classification====
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! Prevalence
! Prevalence
! Origin/definition
! Origin/definition
! Notes/subclassification
! Notes
|-
|-
| Intra-ampullary papillary-tubular carcinoma  
| Intra-ampullary papillary-tubular carcinoma  
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| Peri-ampullary duodenal carcinoma  
| Peri-ampullary duodenal carcinoma  
| ~5% of cases
| ~5% of cases
| primarily in the duodenal, ampullary orfice must be clearly within lesion
| primarily in the duodenum; ampullary orfice must be clearly within lesion
| subclassifiction: (1) intestinal type, (2) mixed mucinous-intestinal type
|  
|-
|-
| Ampullary carcinoma not otherwise specified  
| Ampullary carcinoma not otherwise specified  
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#Pancreaticobiliary ampullary carcinoma.
#Pancreaticobiliary ampullary carcinoma.
#Other.
#Other.
[[File:1 amp ca 1 680x512px.tif|Invasive carcinoma of duodenal ampulla.]]
[[File:2 amp ca 1 680x512px.tif|Invasive carcinoma of duodenal ampulla.]]
<br>
[[File:3 amp ca 1 680x512px.tif|Invasive carcinoma of duodenal ampulla.]]
[[File:4 amp ca 1 680x512px.tif|Invasive carcinoma of duodenal ampulla.]]
<br>
[[File:5 amp ca 1 680x512px.tif|Invasive carcinoma of duodenal ampulla.]]
[[File:6 amp ca 1 680x512px.tif|Invasive carcinoma of duodenal ampulla.]]
Invasive carcinoma of duodenal ampulla, intestinal type. A. Ulceration tops Brunner’s glands [green arrow]; at the edge lie glands [blue arrow] with changes suggesting adenoma (100X). B. Ki67 stain establishes adenoma by surface extension of brown nuclei (100X). C. The lesion was a mass, prompting rebiopsy. The cauterized fragment shows disorderly spread of glands, with dilated glands at base [arrows] not readily explained by obstruction (40X)  D.Two cancerous prongs, one on left [black arrow], one on right [blue arrow] each show spread about muscle fibers (200X). E. The Whipple resection showed the same dilated spreading glands [arrows] at base, redolent of the spread of some well-differentiated colonic adenocarcinomas through muscle (40X). F. Cribriformed cancerous nests [arrows] cannot be Brunner’s glands, because the nuclei are too variable and lack polarity and because they abut crypts extending to the surface; cautery would have made this an impossible distinction (200X).
[[File:5 08466787961551 sl 1.png| 60 year old woman with poorly differentiated ampullary adenocarcinoma and obstructive cholestasis in liver]]
[[File:5 08466787961551 sl 2.png| 60 year old woman with poorly differentiated ampullary adenocarcinoma and obstructive cholestasis in liver]]
[[File:5 08466787961551 sl 3.png| 60 year old woman with poorly differentiated ampullary adenocarcinoma and obstructive cholestasis in liver]]
[[File:5 08466787961551 sl 4.png| 60 year old woman with poorly differentiated ampullary adenocarcinoma and obstructive cholestasis in liver]]
[[File:5 08466787961551 sl 5.png| 60 year old woman with poorly differentiated ampullary adenocarcinoma and obstructive cholestasis in liver]]
[[File:5 08466787961551 sl 6.png| 60 year old woman with poorly differentiated ampullary adenocarcinoma and obstructive cholestasis in liver]]
[[File:5 08466787961551 sl 7.png| 60 year old woman with poorly differentiated ampullary adenocarcinoma and obstructive cholestasis in liver]]
60 year old woman with poorly differentiated ampullary adenocarcinoma and obstructive cholestasis in liver. A. An isolated tumor fragment (black arrow) and two fragments with tumor undermining small intesatinal mucosa (green arrows) are seen. B. Large, ovoid cancer nuclei with abundant grey cytoplasm form sheets with only rare acini (arrow). C. Tumor cells are CK7 positive. D. Tumor cells are CK20 negative. E. Portal triads (black arrows) are expanded, lacking intense inflammation. A septal duct (green arrow) lacks definite onion skinning. F. Proliferated peripheral bile ductules (black arrows) sometimes have neutrophils (white arrow), which should not be interpreted as ascending cholangitis. Next to the artery (red arrow) lies the bile duct (yellow arrow), which has disturbed nuclei, but no neutrophils. Note occasional eosinophils (cyan arrows). G. Hepatocytes with feathery degeneration. Arrows point to dilated cholangioles, one filled with a bile plug. 


Notes:
Notes:
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*Ectopic pancreas.<ref name=pmid18304504>{{Cite journal  | last1 = Hsu | first1 = SD. | last2 = Chan | first2 = DC. | last3 = Hsieh | first3 = HF. | last4 = Chen | first4 = TW. | last5 = Yu | first5 = JC. | last6 = Chou | first6 = SJ. | title = Ectopic pancreas presenting as ampulla of Vater tumor. | journal = Am J Surg | volume = 195 | issue = 4 | pages = 498-500 | month = Apr | year = 2008 | doi = 10.1016/j.amjsurg.2007.01.043 | PMID = 18304504 }}
*Ectopic pancreas.<ref name=pmid18304504>{{Cite journal  | last1 = Hsu | first1 = SD. | last2 = Chan | first2 = DC. | last3 = Hsieh | first3 = HF. | last4 = Chen | first4 = TW. | last5 = Yu | first5 = JC. | last6 = Chou | first6 = SJ. | title = Ectopic pancreas presenting as ampulla of Vater tumor. | journal = Am J Surg | volume = 195 | issue = 4 | pages = 498-500 | month = Apr | year = 2008 | doi = 10.1016/j.amjsurg.2007.01.043 | PMID = 18304504 }}
</ref>
</ref>
*[[Duodenal adenocarcinoma]] with secondary involvement of the ampulla.


====Intestinal ampullary carcinoma====
====Intestinal ampullary carcinoma====
Features:<ref name=pmid23026934/>
Features:<ref name=pmid23026934/>
*Pseudostratified columnar epithelium with hyperchromatic, ellipsoid nuclei.
*Pseudostratified columnar epithelium with hyperchromatic, ellipsoid nuclei.
**Similar to [[colorectal adenocarcinoma]].
**Glands usually tightly packed, i.e. high gland-to-stroma ratio; often >2:1.
 
Note:
*Similar to [[colorectal adenocarcinoma]].


====Pancreatobiliary ampullary carcinoma====
====Pancreatobiliary ampullary carcinoma====
Features:<ref name=pmid23026934/>
Features:<ref name=pmid23026934/>
*Tubular arrangements consisting of cuboidal cells in one or two layers.
*Tubular arrangements consisting of cuboidal cells in one or two layers.
**Tubules usually spaced; ~1:3 gland-to-stroma ratio.
=====Images=====
<gallery>
Image: Periampullary adenocarcinoma -- low mag.jpg | PA - low mag. (WC)
Image: Periampullary adenocarcinoma -- intermed mag.jpg | PA - intermed. mag. (WC)
Image: Periampullary adenocarcinoma -- high mag.jpg | PA - high mag. (WC)
Image: Periampullary adenocarcinoma - alt -- high mag.jpg | PA - high mag. (WC)
</gallery>


====Other====
====Other====
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*#Mixed-mucinous.
*#Mixed-mucinous.
*#Mucinous-signet-ring.
*#Mucinous-signet-ring.
<gallery>
Image:Ampulla AmpullaryACA-2 PA.JPG|Ampulla Ampullary Adeno Carcinoma -(SKB)
Image:Ampulla AmpullaryACA-3 PA.JPG|Ampulla Ampullary Adeno Carcinoma - (SKB)
Image:Ampulla AmpullaryACA PA.JPG|Ampulla Ampullary Adeno Carcinoma - (SKB)
</gallery>


===IHC===
===IHC===
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*SMAD4 +ve/-ve.
*SMAD4 +ve/-ve.
**Lost in pancreatic neoplasia ~90% of cases vs. ~35% of ampullary tumours.<ref name=pmid12640108>{{Cite journal  | last1 = McCarthy | first1 = DM. | last2 = Hruban | first2 = RH. | last3 = Argani | first3 = P. | last4 = Howe | first4 = JR. | last5 = Conlon | first5 = KC. | last6 = Brennan | first6 = MF. | last7 = Zahurak | first7 = M. | last8 = Wilentz | first8 = RE. | last9 = Cameron | first9 = JL. | title = Role of the DPC4 tumor suppressor gene in adenocarcinoma of the ampulla of Vater: analysis of 140 cases. | journal = Mod Pathol | volume = 16 | issue = 3 | pages = 272-8 | month = Mar | year = 2003 | doi = 10.1097/01.MP.0000057246.03448.26 | PMID = 12640108 }}</ref>
**Lost in pancreatic neoplasia ~90% of cases vs. ~35% of ampullary tumours.<ref name=pmid12640108>{{Cite journal  | last1 = McCarthy | first1 = DM. | last2 = Hruban | first2 = RH. | last3 = Argani | first3 = P. | last4 = Howe | first4 = JR. | last5 = Conlon | first5 = KC. | last6 = Brennan | first6 = MF. | last7 = Zahurak | first7 = M. | last8 = Wilentz | first8 = RE. | last9 = Cameron | first9 = JL. | title = Role of the DPC4 tumor suppressor gene in adenocarcinoma of the ampulla of Vater: analysis of 140 cases. | journal = Mod Pathol | volume = 16 | issue = 3 | pages = 272-8 | month = Mar | year = 2003 | doi = 10.1097/01.MP.0000057246.03448.26 | PMID = 12640108 }}</ref>
*p53 +ve ~55% of cases.<ref name=pmid11144926>{{Cite journal  | last1 = Takashima | first1 = M. | last2 = Ueki | first2 = T. | last3 = Nagai | first3 = E. | last4 = Yao | first4 = T. | last5 = Yamaguchi | first5 = K. | last6 = Tanaka | first6 = M. | last7 = Tsuneyoshi | first7 = M. | title = Carcinoma of the ampulla of Vater associated with or without adenoma: a clinicopathologic analysis of 198 cases with reference to p53 and Ki-67 immunohistochemical expressions. | journal = Mod Pathol | volume = 13 | issue = 12 | pages = 1300-7 | month = Dec | year = 2000 | doi = 10.1038/modpathol.3880238 | PMID = 11144926 }}</ref><ref>{{Cite journal  | last1 = Park | first1 = SH. | last2 = Kim | first2 = YI. | last3 = Park | first3 = YH. | last4 = Kim | first4 = SW. | last5 = Kim | first5 = KW. | last6 = Kim | first6 = YT. | last7 = Kim | first7 = WH. | title = Clinicopathologic correlation of p53 protein overexpression in adenoma and carcinoma of the ampulla of Vater. | journal = World J Surg | volume = 24 | issue = 1 | pages = 54-9 | month = Jan | year = 2000 | doi =  | PMID = 10594204 }}</ref>
*E-cadherin +ve ~40% of cases.<ref name=pmid16506371/>
*Beta-catenin +ve ~65% of cases.<ref name=pmid16506371>{{Cite journal  | last1 = Park | first1 = S. | last2 = Kim | first2 = SW. | last3 = Lee | first3 = BL. | last4 = Jung | first4 = EJ. | last5 = Kim | first5 = WH. | title = Expression of E-cadherin and beta-catenin in the adenoma-carcinoma sequence of ampulla of Vater cancer. | journal = Hepatogastroenterology | volume = 53 | issue = 67 | pages = 28-32 | month =  | year =  | doi =  | PMID = 16506371 }}
</ref>


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