Adenocarcinoma of Papilla of Vater
Adenocarcinoma of Papilla of Vater

Video Endoscopic Sequence 1 of 5.

Adenocarcinoma of Papilla of Vater

This 68-year-old male was referred to our unit for evaluation of icteric syndrome.

Carcinoma of the ampulla of Vater is a malignant tumor arising in the last centimeter of the common bile duct, where it passes through the wall of the duodenum and ampullary papilla. Treatment fails in nearly 70% of patients with poor prognostic features.

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Adenocarcinoma of Papilla of Vater

Video Endoscopic Sequence 2 of 5.

Adenocarcinoma of Papilla of Vater

A stent was placed

The standard surgical approach to the treatment of ampullary carcinoma is pancreaticoduodenal resection (Whipple procedure). The procedure involves en bloc resection of the gastric antrum and duodenum; a segment of the first portion of the jejunum, gallbladder, and distal common bile duct; the head and often the neck of the pancreas; and adjacent regional lymph nodes.

The operative mortality rate for pancreaticoduodenectomy was at one time reported to be approximately 20%, but several hospital centers have since reported large series with operative mortality rates in the range of 5%.

 

 

Adenocarcinoma of Papilla of Vater

Video Endoscopic Sequence 3 of 5.

Adenocarcinoma of Papilla of Vater

Adenoma-carcinoma sequence also applies to the papill of Vater and to determine the frequency with which a tumour the papilla of Vater, diagnosed histologically as containing portions of an adenoma, already contains adenocarcinoma elsewhere.

 

Adenocarcinoma of Papilla of Vater

Video Endoscopic Sequence 4 of 5.

Adenocarcinoma of Papilla of Vater

Ampullary tumors are usually diagnosed during endoscopic evaluation for symptoms or at routine surveillance of FAP patients. A side-viewing duodenoscope is optimal for assessing for ampullary lesions. Ampullary adenomas may be polypoid, fungating, ulcerated, or sessile goatee appearance particularly in FAP.

 

Adenocarcinoma of Papilla of Vater

Video Endoscopic Sequence 5 of 5.

Adenocarcinoma of Papilla of Vater

Benign tumors of the ampulla of Vater, or major duodenal papilla, can be treated with endoscopic resection termed papillectomy or ampullectomy with or without adjunctive ablative therapy. Ampullary adenomas occur in 0.04 to 0.12 % of the general population and in 50% to 100% of persons with familial adenomatous polyposis (FAP). These lesions can progress through an adenoma-carcinoma sequence, as do colorectal adenomas. Ampullary carcinomas are a frequent cause of death in persons with FAP.

Pancreaticoduodenectomy or local surgical resection have been the traditional treatment. However, perioperative mortality occurs in 4% to 15% and morbidity in up to 50% following pancreaticoduodenectomy. Local resection has a morbidity rate of 19% to 25% with recurrence in up to 32% at 5 years.

Endoscopic therapy entails snare resection of ampullary adenomas with or without ablation with ionized argon coagulation, bipolar or heat probe, or Nd:YAG laser. Photodynamic therapy has also been used. Frequently, a combination of mechanical and thermal therapy is necessary and there is no universally accepted technique.

 

Adenocarcinoma of the Gallbladder.

Video Endoscopic Sequence 1 of 4.

Adenocarcinoma of the Gallbladder.

Adenocarcinoma of the gallblader that infiltrates the duodenal walls.
A 53 year-old female with adenocarcinoma of the gallblader.

 

Adenocarcinoma of the Gallbladder.

Video Endoscopic Sequence 2 of 4.

Adenocarcinoma of the Gallbladder.

A biliar stent 12 Fr. was placed in the Vater papilla.

 

Adenocarcinoma of the Gallbladder.

Video Endoscopic Sequence 3 of 4.

Adenocarcinoma of the Gallbladder.

Due to recurrent episodic bleeding, successfully used of argon plasma coagulation (APC) is performed as a palliative treatment.

 

Adenocarcinoma of Papilla of Vater. Duodenoscopic view

Video Endoscopic Sequence 4 of 4.

Adenocarcinoma of Papilla of Vater. Duodenoscopic view.

 

Carcinoma of the papilla of Vater.

Adenocarcinoma of the papilla of Vater.

This image and the video is observed through a
forward-viewing endoscope. 

 

Villous adenoma Adenoma of Vater Papilla and Juxtapapillary diverticula.

Villous adenoma Adenoma of Vater Papilla and Juxtapapillary diverticula.

This is the case of a seventy-three year old male with a biopsy proven ampullary villous adenoma, presented with jaundice, showed markedly elevated alkaline fosfatase and the abdominal ultrasound displays a dilated biliary tree.

Adenomas of the papilla of Vater are relatively rare tumours. They are of particular interest, not only because of their particular topography, but also because the adenoma-carcinoma sequence - accepted in the colorectum has also been postulated to apply to the papilla of Vater. In fact, ampullary adenoma is often considered to be a precancerous lesion.

 

Adenocarcinoma of Papilla of Vater

Video Endoscopic Sequence 1 of 5.

Adenocarcinoma of Papilla of Vater

Forward-viewing gastroscope.

 

Adenocarcinoma of Papilla of Vater

Video Endoscopic Sequence 2 of 5.

Adenocarcinoma of Papilla of Vater. Duodenoscopic view.

 

Adenocarcinoma of Papilla of Vater

Video Endoscopic Sequence 3 of 5.

Carcinoma of the ampulla of Vater is a malignant tumor arising within 2 cm of the distal end of the common bile duct, where it passes through the wall of the duodenum and ampullary papilla. The common bile duct merges with the pancreatic duct of Wirsung at this point and exits through the ampulla into the duodenum. The most distal portion of the common bile duct is dilated (ie, forms the ampulla of Vater) and is surrounded by the sphincter of Oddi, which spirals upward around the terminal portion of the duct. Because of biliary outflow obstruction, carcinoma of the ampulla of Vater tends to manifest early, as opposed to other pancreatic neoplasms that often are advanced at the time of diagnosis.

 

Adenocarcinoma of Papilla of Vater

Video Endoscopic Sequence 4 of 5.

Adenocarcinoma of Papilla of Vater

Chromoendoscopy with indigo carmin stain.

Curative surgical resection is the only option for long-term survival. Surgical or radiologic biliary decompression, relief of gastric outlet obstruction, and adequate pain control may improve the quality of life but do not affect overall survival rate.

 

Video Endoscopic Sequence 5 of 5.

Image of Adenocarcinoma of Papilla of Vater

Lymph nodes metastases are present in as many as half of patients. Pericanalicular lymph nodes usually are the first to be involved. Nodes along the superior mesenteric, gastroduodenal, common hepatic, and splenic arteries, as well as the celiac trunk, are the second station of lymph nodes. Perineural, vascular, and lymphatic invasion are associated with a poor prognosis. Liver is the most common site (66%) of distant metastasis, followed by lymph nodes (22%). In advanced cases, lung metastasis also may occur.

 

Adenocarcinoma of the Gallbladder

Video Endoscopic Sequence 1 of 3.

Adenocarcinoma of the Gallbladder

Adenocarcinoma of the gallblader that infiltrates the duodenal walls.
A 53 year-old female with adenocarcinoma of the gallblader.

 

Adenocarcinoma of the Gallbladder

Video Endoscopic Sequence 2 of 3.

Adenocarcinoma of the Gallbladder

A biliar stent 12 Fr. was placed in the Vater papilla.

 

Video Endoscopic Sequence 3 of 3.

Adenocarcinoma of the Gallbladder

Due to recurrent episodic bleeding, successfully used of argon plasma coagulation (APC) is performed as a palliative treatment.

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 1 of 20.

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula and the head of the pancreas.

This 77 year-old male, presented with silent jaundice, he
has been under medical check up with an upper endoscopy,
Computed Tomography and different Imaging Studies in
another institution but the diagnosis was overlooked.

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 2 of 20.

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Cholangiocarcinomas (CCCs) are malignancies of the biliary duct system that may originate in the liver and extrahepatic bile ducts, which terminate at the ampulla of Vater. CCCs are encountered in 3 geographic regions: intrahepatic, extrahepatic (ie, perihilar), and distal extrahepatic. Perihilar tumors are the most common, and intrahepatic tumors are the least common. Perihilar tumors, also called Klatskin tumors (after Klatskin's description of them in 1965), occur at the bifurcation of right and left hepatic ducts.

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 3 of 20.

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

 

 

 

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 4 of 20.

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

 

 

 

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 5 of 20.

Macroscopic view of Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula


 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 6 of 20.

Macroscopic view of Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Clinical Features

Most common presenting clinical features of perihilar or extrahepatic tumours are those of biliary obstruction: jaundice, pale stool, dark urine, and pruritus.

Right upper quadrant pain, fever, and rigors suggest cholangitis (this is unusual without drainage attempts).

Cholangiocarcinoma usually presents after the disease is advanced. This is particularly true with more proximal intrahepatic and perihilar tumours obstructing one duct, which often present with systemic manifestations of malignancy, such as malaise, fatigue, and weight loss.

Some cases are detected incidentally as a result of deranged liver function tests, or ultrasound scans performed for other indications.

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 7 of 20.

Macroscopic view of Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 8 of 20.

Distal choledoco duct tumor pushing and distorting adjacent duodenal diverticulum.

Cholangiocarcinoma and bile duct cancer are often used interchangeably. Primary biliary tract malignancies. More than 95% of these malignancies are cholangiocarcinomas (epithelial adenocarcinomas ) frequently found in the extrahepatic biliary tree.

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 9 of 20.

Close up of cholangiolar tumor and duodenal diverticulum

The tumor is usually small and may arise anywhere along
the biliary tree, from the small intrahepatic bile ducts to the
common bile duct. Microscopically, cholangiocarcinoma
may resemble adenocarcinoma. These bile ductale tumors
may be well differentiated, while others are poorly
differentiated.

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 10 of 20.

Tumor with pancreatic tissue at the bottom

Cholangiocarcinomas are usually slow-growing tumors that
spread locally via the lymphatic system. Treatment and
long-term prognosis are dependent upon the location of the
mass. Lesions located in the distal or middle portion of the
extrahepatic bile duct (20% and 35%, respectively) have a
better prognosis than tumors in the proximal third, which
include about 45% of bile duct cancers (including
Klatskin’s tumors — hilar variants).

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 11 of 20.

An inverted picture of tumor showing the duodenal,
diverticular and pancreatic relations

Large solitary tumors are characteristic of peripheral
cholangiocarcinoma; however, a multinodular type may
occur. These tumors have a fibrous stroma, are firm and
grayish white in color, and are not well vascularized. Hilar
cholangiocarcinoma are usually firm, intramural, annular
tumors that encircle the bile duct, or may be bulky hard
masses that are on the duct or hilar region and extend into
the liver. They may also appear as a spongy friable mass
in the lumen of the bile duct. There may be metastatic
nodules throughout the liver with dilation of bile ducts
peripheral to the mass.

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 12 of 20.

Diverticular opening and tumor invading the ampullar
region. There was not possible identification of the
ampullar openig.

WHO classification of carcinomas of the liver

Hepatocellular carcinoma

Combined hepatocellular cholangiocarcinoma

Cholangiocarcinoma, intrahepatic

Bile duct cystadenocarcinoma

Undifferentiated carcinoma

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 13 of 20.

Macroscopic view of Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Complete tumoral obstruction of the distal choledoco the duodenal diverticulum is located to the right.

The incidence of biliary cancers corresponds to mortality rates as the prognosis from these tumours is very poor.

 

Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

Video Endoscopic Sequence 14 of 20.

Macroscopic view of Cholangiocarcinoma that infiltrated a Periampullary Duodenal Diverticula

WHO classification of carcinomas of the extrahepatic bile ducts.

Carcinoma in situ

Adenocarcinoma

Papillary adenocarcinoma

Adenocarcinoma, intestinal-type

Mucinous adenocarcinoma

Clear cell adenocarcinoma

Signet ring cell carcinoma

Adenosquamous carcinoma

Squamous cell carcinoma

Small cell carcinoma (oat cell carcinoma)

Undifferentiated carcinoma.

 

Video Endoscopic Sequence 15 of 20.

Neoplastic tubulopapillar tissue infiltrating and replacing the Vater ampulla.

Histological Grade

Most cholangiocarcinomas (95%) are adenocarcinomas.

Adenocarcinomas are classified 1 to 4 according to the percentage of tumour that is composed of glandular tissue.

Some types of adenocarcinoma are however not graded: carcinoma in situ, clear cell adenocarcinoma, and papillary adenocarcinoma.

Signet ring cell carcinoma is given a grade of 3 and small cell.

Carcinoma a grade of 4. Squamous cell carcinomas are graded according to the least differentiated areas. Most studies have demonstrated a relation between histological grade and postoperative outcome although stage is more important.

 

Video Endoscopic Sequence 16 of 20.

Large and small malignant cholangiolar ducts

Anatomical classification “Cholangiocarcinoma” originally
referred only to primary

tumours of the intrahepatic bile ducts and was not used for extrahepatic bile duct tumours but the term is now regarded as inclusive of intrahepatic, perihilar, and distal extrahepatic tumours of the bile ducts. 20–25% are intrahepatic. 50–60% of all cases of cholangiocarcinoma are perihilar tumours (those involving the bifurcation of the ducts are “Klatskin” tumours).

Most Klatskin tumours may have been coded as intrahepatic

tumours for purposes of death certification.

20–25% are distal extrahepatic tumours.

About 5% of tumours may be multifocal.

The extent of duct involvement by perihilar tumoursmay be

classified as suggested by Bismuth:

type I: tumours below the confluence of the left and right hepatic ducts;

type II: tumours reaching the confluence but not involving the left or right hepatic ducts;

 

Video Endoscopic Sequence 17 of 20.

Microscopic pattern of the tumor with columnar epithelium and hyperchromatic nuclei.

 

Video Endoscopic Sequence 18 of 20.

Terminal biliar duct with neoplastic small glands proliferation and mucosa of the choledoco with neoplasia.

 

Video Endoscopic Sequence 19 of 20.

Low power detail of tumor invasion to the pancreas head at left superior corner and to the periampullar duodeno at right inferior corner.

 

Video Endoscopic Sequence 20 of 20.

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