Periampullary Carcinoma

The ampulla of Vater is a mound-like opening in the duodenum. The duodenum is a C shaped loop of bowel at the beginning of the intestine. It is where the bile duct bringing bile from the liver and pancreatic duct which brings pancreatic juice from pancreas meets and then opens into the intestine.

Periampullary cancer is a term used to refer to cancer that occurs in the vicinity of the ampulla of Vater. This includes cancer affecting the head of the pancreas, distal bile duct (cholangiocarcinoma), duodenum, and ampulla. These cancers are grouped together because they share similar clinical features and treatment options. Although they are all adenocarcinomas, their prognosis varies. Surgical removal of these tumors, if possible, offers the best chances of a cure.

Periampullary cancer types

Periampullary cancer refers to different types of cancer that occur around the ampulla of Vater. These types of cancer include:

• Pancreatic cancer: a cancer that originates in the pancreas. You can read more about pancreatic cancer here.
• Distal bile duct cancer (cholangiocarcinoma): a cancer that arises from the lining of the lowermost part of the bile duct, where it passes through the pancreas, joins the pancreatic duct, and ends in the intestine.
• Duodenal cancer: a cancer that arises from the lining of the duodenal mucosa. The duodenum is the part of the small intestine where food from the stomach mixes with bile and pancreatic juice. Most duodenal cancers originate in the second part of the duodenum, where the ampulla is located.
• Ampullary cancer: a cancer that arises from the lining of the ampulla of Vater. You can read more about ampullary cancer here .

Risk factors or causes of periampullary cancer

Anything that increases your risk of getting a disease is called a risk factor. The risk of someone getting periampullary cancer increases with age. Another common risk factor is regular smoking and drinking of alcohol. Some patients get this disease because they are genetically more prone. The risk factors are slightly different for all these four cancer types.

Pancreatic cancer: you can read about risk factors for pancreatic cancer here .

Risk factors for bile duct cancer (cholangiocarcinoma) are infestation with some parasites such as liver flukes, primary sclerosing cholangitis, gallstones, choledochal cysts, diabetes and obesity.

Risk factors for duodenal cancer include inherited conditions like Familial adenomatous polyposis (FAP), Gardner syndrome, HNPCC or Lynch syndrome, juvenile polyposis syndrome and Peutz-Jeghers syndrome. Crohn's disease, celiac disease and duodenal polyps also increase the risk. Increased intake of sugar and red meat with reduced intake of fruits and vegetables has also been incriminated.

Risk factors for ampullary cancer have not been defined due to the low incidence of this disease in the population. According to a case-control study, previous cholecystectomy (gallbladder removal) and use of proton pump inhibitors (PPIs) were associated with increased risk of ampullary tumors.

Signs and symptoms

Periampullary cancers compress the bile duct, blocks the flow of bile and cause yellowing of eyes and urine called jaundice. This is usually accompanied by pale stools and itching. Other warning signs of periampullary cancer can be weight loss, loss of appetite and abdominal pain. Besides, duodenal and ampullary cancers bleed causing black stools (melena) and anaemia.

Signs and symptoms of periampullary cancer

• Jaundice (yellowing of skin, eyes and urine with pale stools)
• Itching
• Abdominal pain
• Weight loss and loss of appetite
• Recurrent vomiting
• Black stools
• Anaemia

Diagnosis and staging of periampullary cancer

Once periampullary cancer is suspected, it is diagnosed and staged by a high resolution, thin cut, triple-phase CT scan or PET scan. Your surgeon will also do tests to quantify your jaundice, check your kidney function, haemoglobin and blood clotting parameters. A tumour marker called CA19.9 will also be checked. A chest X-ray or a CT scan of chest will be done to look for any tumour in the chest.

Side viewing endoscopy: it is a procedure in which a thin flexible tube is passed into your intestine through your mouth. It has a camera at the tip of the tube and a view of the ampulla is obtained, showing any cancerous growth.

Biopsy: if a tumour is found at the ampulla, then a small sample from it is obtained called as biopsy and examined under microscope confirming the diagnosis.

Endoscopic ultrasound (EUS): it is like doing an ultrasound of the periampullary region from the inside of your intestine. It aids diagnosis in cases where CT/MRI do not show cancerous growth, while it is suspected on clinical features. It is also used to take a sample from the tumour by passing a needle called fine needle aspiration cytology (FNAC) for confirmation of diagnosis if required.

Treatment of periampullary cancer

The treatment depends upon the stage of the disease and degree of jaundice. Surgery is the best possible option and can be considered if the cancer is diagnosed at a stage where it can be completely removed by surgery. If the jaundice is very high, your surgeon may choose to decrease jaundice before surgery by doing a procedure called endoscopic retrograde cholangiopancreatography (ERCP) and stenting. In this, a plastic or metallic tube called stent is placed in the bile duct which is blocked by tumour and opens it up from inside.

If the tumour is advanced then he may also give you neoadjuvant treatment (chemotherapy or chemoradiotherapy) to decrease the size of the tumour and then resect it to increase your chances of survival.

For unresectable tumours, an attempt to made to downstage them to a stage where they can be surgically removed. For this neoadjuvant treatment is administered and some of these patients will become operable.

Surgery for periampullary cancer

The operation to surgically remove periampullary cancer is called Whipple operation, also known as pancreaticoduodenectomy. In this, head of the pancreas is removed along with duodenum, bile duct, gall bladder, part of the stomach, a small part of the small intestine and adjacent lymph nodes. To restore gastrointestinal continuity, the small intestine is then joined to the pancreas (sometimes pancreas is joined to the stomach), remaining bile duct and stomach.

Palliative treatment

Chemotherapy uses drugs to destroy cancer cells. For cancers which have spread to distant organs of the body (metastatic), surgery is not an option. After FNAC/biopsy and stenting (if jaundiced) chemotherapy is given.

Endoscopic retrograde cholangiopancreatography (ERCP) and stenting: In this, a plastic or metallic tube called stent is placed in the bile duct which is blocked by tumour and opens it up from inside alleviating jaundice.

If the tumour is causing blockage of food passage then a metal stent is placed in the food passage endoscopically. If this fails then a bypass surgery will be required.

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