Tumour of Ampulla of Vater

Tumour of the ampulla of Vater is a rare cancer that develops in a small but crucial opening where bile and digestive juices from your liver and pancreas meet before emptying into your small intestine. Although rare, affecting less than 1% of digestive system cancers, early detection can make a significant difference in treatment outcomes.

Table of contents

• What is a tumour of the ampulla of Vater?
• How common is this cancer?
• What causes this cancer?
• Who is at risk?
• Signs and symptoms
• How is it diagnosed?
• Treatment options
• Outlook and survival

What is a tumour of the ampulla of Vater?

A tumour of the ampulla of Vater is a rare type of cancer that develops in a small opening called the ampulla of Vater. This opening is located where two important tubes join together: the bile duct (which carries bile from your liver) and the pancreatic duct (which carries digestive juices from your pancreas). These tubes meet at the ampulla of Vater and empty their contents into the first part of your small intestine, called the duodenum^[1][2].

The ampulla is lined by a layer of tissue called epithelial tissue. Most cancers of the ampulla start in epithelial cells that keep the tissue moist. Doctors call these cancers adenocarcinomas. The epithelial cells in the ampulla can be similar to cells that line the duodenum, or they might be like the cells lining the pancreas and common bile duct. Sometimes the cells might be a mix of both^[9].

Because the ampulla of Vater is located close to the liver, pancreas, and other digestive organs, cancer that forms there can easily spread to these nearby areas^[2].

ampullary cancer, ampullary carcinoma, ampulla of Vater cancer

How common is this cancer?

Ampullary cancer is very rare. It accounts for less than 1% of all cancers affecting the digestive system^[2]. The incidence rate is approximately 0.49 per 100,000 individuals, though this rate has been increasing at an annual percentage rate of 0.9%^[5]. In the UK, around 550 people are diagnosed with ampullary cancer each year^[9].

What causes this cancer?

Experts aren’t sure exactly what causes ampullary cancer. Like other cancers, it occurs when cells in the body develop changes in their DNA (the instructions that tell cells what to do). In healthy cells, DNA gives instructions to grow and multiply at a set rate and tells the cells to die at a set time^[1].

In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to make many more cells quickly and to keep living when healthy cells would die. These abnormal cells may grow to form a lump or mass called a tumor. If they are in the body long enough, they can grow into nearby areas and spread to other parts of the body, a process called metastasis^[3][2].

Experts continue to learn more about the specific types of DNA changes associated with ampullary cancer. This information helps them develop treatments that target the abnormalities and kill cancer cells, but this research is still in its early stages^[2].

Who is at risk?

There are no known clear risk factors for ampullary cancer. However, you may have a possible increased risk if you^[3][6]:

• Are an older adult, particularly over the age of 70^[2]
• Are male^[2]
• Have certain inherited health problems that cause growths (called polyps) in the digestive system, such as familial adenomatous polyposis (FAP), Lynch syndrome, or Peutz-Jeghers syndrome^[2][9]
• Have inflammatory bowel disease^[3]

These factors may increase your risk, but having one or more risk factors doesn’t mean you will definitely develop cancer. Some risk factors may not be in your control, but others may be things you can change^[6].

Signs and symptoms

The most common symptom of ampullary cancer is jaundice, which is yellowing of the skin and the whites of the eyes. This happens because the tumor in the ampulla of Vater blocks the bile duct. When bile flow is blocked from going into the intestines, it goes into the blood instead, causing the yellow color in your skin and eyes^[3][6].

Jaundice can also cause^[9]:

• Light colored poo
• Dark colored urine
• Itchy skin
• A high temperature (fever), which may make you feel cold and shivery

Other symptoms of ampullary cancer include^[1][2][3]:

• Unexplained weight loss
• Loss of appetite
• Abdominal pain (pain in your belly)
• Back pain
• Nausea and vomiting
• Diarrhea (loose poo)
• Fever
• Blood in your poo or sick (vomit)
• Low red blood cell counts (anemia)
• Inflammation of the pancreas (pancreatitis), which can cause you to have a fever, feel or be sick, and have pain in your upper belly that may go around to your back
• Pale, greasy stools
• Fatigue

Many of these symptoms may be caused by other health problems that are much more common than ampullary cancer. However, it’s important to see your healthcare provider if you have persistent symptoms that worry you^[3].

You should see your GP urgently or call NHS 111 if you have symptoms of pancreatitis or jaundice, or you had blood in your poo or sick but it has now stopped and you have no other symptoms. Go to A&E or call 999 if you have bleeding from your bottom that isn’t stopping or there is lots of blood, or you have blood in your sick and have black poo or feel unwell^[9].

How is it diagnosed?

Because jaundice is the most common symptom of ampullary cancer, yellowing of the skin is often what leads people to see a healthcare provider. Your provider will ask you about your health history, symptoms, risk factors, and family history of disease. They will also do a physical exam^[3][6].

You may have one or more of these tests^[3][10]:

• Blood and urine tests: These are used to look for anemia, bilirubin levels (a substance that causes jaundice), and other changes that may be signs of this cancer. Blood tests may also check for tumor markers like CA 19-9^[8].
• Imaging tests: These can be used to look for a tumor inside the ampulla of Vater. They may include ultrasounds, CT scans (computed tomography), or MRI scans (magnetic resonance imaging). A special type of MRI called magnetic resonance cholangiopancreatography (MRCP) may be used^[2].
• Endoscopy: Healthcare providers may use an endoscope (a long, thin tube with a tiny video camera on the end) to look at the ampulla. The endoscope is threaded down the mouth, through the esophagus and stomach, and into the duodenum to the ampulla^[3][10].
• Endoscopic ultrasound: This combines endoscopy with ultrasound to capture detailed images of the ampullary cancer^[10].
• Endoscopic retrograde cholangiopancreatography (ERCP): This test uses an endoscope to inject a dye into the bile duct. The dye shows up on X-rays and can help look for blockages in the bile duct or pancreatic duct^[3][10].
• Biopsy: This involves collecting a small tissue sample that a specialist called a pathologist can test for cancer cells. Providers often collect the sample during an endoscopic test. A biopsy is the only way to confirm this cancer^[2][3].

After a diagnosis of ampullary cancer, you’ll likely need more tests to help your healthcare providers learn more about the cancer. These tests can help find out the stage of cancer, which is how much and how far the cancer has spread in your body. The stage is one of the most important things to know when deciding on treatment^[3].

Treatment options

The main treatment for ampullary cancer is surgery to remove the cancer. Treatment may also include chemotherapy and radiation therapy^[1].

Surgery

Surgical removal of the cancer is the primary treatment and the best option for a cure. The most common operation is called pancreaticoduodenectomy, also known as the Whipple procedure. This surgery removes the head of the pancreas along with the affected area. Five-year survival after surgery ranges between 10% and 75% depending on the stage of the cancer^[11][8].

For some patients who may be unable to tolerate major surgery, a smaller operation called local excision may be an option^[8].

Surgery is not possible if the cancer has spread to the liver, has caused growths in the abdominal lining, involves major blood vessels, or has spread to lymph nodes outside the area that can be removed^[15].

Chemotherapy and radiation therapy

The role of chemotherapy and radiation therapy after surgery (called adjuvant therapy) remains controversial. Some studies suggest that chemotherapy with drugs like 5-fluorouracil or capecitabine, sometimes combined with radiation therapy, may improve survival for certain patients, particularly those with lymph node involvement or advanced tumor stages. However, there is not enough scientific evidence to make firm recommendations^[11][15].

For patients with cancer that cannot be removed by surgery or has spread to other parts of the body (metastatic disease), chemotherapy may be used as the main treatment. Treatment approaches may be similar to those used for cancer of the colon, pancreas, or bile duct^[11][16].

Therapeutic decisions should be discussed in a team of different specialists (called an interdisciplinary tumor board) and should take into account the specific characteristics of the cancer^[11].

Outlook and survival

Ampullary cancer has a poor outlook overall, but patients with this cancer generally have better survival than patients with pancreatic cancer. The five-year survival rate is 41% to 45% for cancer that is confined to the local area, but only 4% to 7% for cancer that has spread to other parts of the body^[11].

The outlook depends on several factors, including the stage of the cancer and certain features seen under the microscope. Most ampullary cancers fall into two main subtypes based on how the cells look: intestinal type or pancreaticobiliary type. Patients with the pancreaticobiliary subtype tend to have shorter survival times, with median survival of 33 to 41 months compared to 72 to 80 months for the intestinal type^[5][11].

Treating ampullary cancer with surgery before it spreads offers the best outlook for the disease^[2].