Ampullary cancer is a rare form of cancer that develops in the ampulla of Vater. This is a small opening located on the duodenum, which is the first section of the small intestine. Secretions from the bile ducts and pancreas enter the intestines through this opening.

People can sometimes confuse ampullary cancer with periampullary cancer, which develops nearby.

This article looks at ampullary cancer in more detail, including its symptoms, causes, and treatments. It also discusses a person’s potential outlook and considers means of support.

A medical professional checking the results of the tests for ampullary cancer.Share on Pinterest
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Ampullary cancer is a relatively rare form of cancer. It accounts for about 6% of all cancers that develop in the periampullary area.

It develops on the ampulla of Vater, which is a tiny opening located on the duodenum. This opening allows secretions from the pancreas and bile ducts to pass through to aid in the digestion of food.

It is not the same as periampullary cancers, which start in the areas around the ampulla of Vater.

The most common symptom associated with ampullary cancer is obstructive jaundice. As the cancer grows, it can block the bile ducts and prevent the bile from entering the small intestine.

Obstructive jaundice usually presents as yellowing of the eyes and skin alongside pale and foul-smelling stool and dark urine. In some cases, people may also have itchy skin.

Other possible symptoms of ampullary cancer include:

Some of these symptoms occur when ampullary cancer affects nearby organs, such as the small intestine, liver, and pancreas.

According to a 2022 article, experts believe that many cases of ampullary cancer occur sporadically.

However, they suspect that the risk of developing the cancer is 200 times higher among people with a genetic predisposition to the condition, such as those with hereditary polyposis syndrome or hereditary nonpolyposis colorectal cancer.

Doctors tend to diagnose people when they are in their 60s.

Can a person prevent ampullary cancer?

There are no known risk factors for ampullary cancer, which means that a person cannot take steps to prevent it from occurring.

As ampullary cancer is a rare form of cancer, there is limited research on how to treat it. As a result, doctors do not have a lot of guidance regarding the treatment options.

When they catch the cancer early, a doctor may recommend a combination of surgery and chemotherapy. The surgical procedure, called pancreaticoduodenectomy or the Whipple procedure, removes the:

  • duodenum
  • head of the pancreas
  • bile duct
  • gallbladder

Following the surgery, a doctor will typically recommend chemotherapy with a drug called gemcitabine. This helps remove any remaining cancer cells.

For more advanced cancer that has spread to other organs, a 2014 article states that a doctor will likely recommend systemic treatment.

Systemic treatment involves a combination of chemotherapy medications, such as fluoropyrimidine or gemcitabine, with a platinum compound — often cisplatin or oxaliplatin. These medications can help prevent the cancer from spreading and potentially reduce the symptoms.

Doctors may also suggest systemic treatment if they do not feel as though a person can safely undergo surgery.

The survival rate refers to the proportion of people who are still alive for a length of time after receiving a particular diagnosis. For example, a 5-year survival rate of 50% means that 50%, or half, of those who receive a certain diagnosis are still alive 5 years afterward.

Researchers note that in the early stages, ampullary cancer has a relatively high survival rate. The average 5-year survival rate for ampullary cancer is in the range of 70–80% for localized cancer. If the cancer has spread to the lymph nodes, this figure drops to 20–50%.

People often receive treatment in the earlier stages of the cancer, as it produces symptoms quickly. However, following treatment, the cancer will return in approximately 45% of people.

A doctor will likely recommend chemotherapy for people with larger tumors or cancer that has spread.

It is important to remember that these figures are estimates based on the results of previous studies or treatments. A person can consult a healthcare professional about how their condition will likely affect them.

Diagnosis may be difficult. This is because ampullary cancer resembles several other local cancers, including pancreatic cancer, distal biliary ductal cancer, and colon cancer.

A doctor will likely use a few tests to help make a diagnosis. The tests can include:

  • endoscopic ultrasound
  • endoscopic retrograde cholangiopancreatography
  • fine-needle aspiration cytology

The diagnostic process also involves staging the cancer. To do this, a doctor will typically order a CT scan of the abdomen, chest, and pelvis.

Stages of ampullary cancer

There are four stages of ampullary cancer:

  • Stage 1: The tumor has developed on the epithelium. This is the tissue that covers the internal and external surfaces of the body. It also lines the cavities and hollow organs in the body.
  • Stage 2: The tumor has spread to the duodenal submucosa, which is the middle layer of the small intestine. The tumor may also affect the sphincter of Oddi, which is a valve in the digestive tract that helps move the bile and juices from the pancreas into the small intestine.
  • Stage 3: The tumor has affected the duodenal muscularis propria, which is the outer wall of the intestine.
  • Stage 4: The cancer has spread to other areas of the body, such as the lymph nodes. It may have also spread to other organs, such as the pancreas or the liver.

Ampullary cancer is rare, meaning that few specific support groups are available. A person may find it helpful to join a group of pancreatic cancer survivors. They can search for pancreatic cancer groups using the following resources:

Certain lifestyle adjustments can also promote better physical and mental well-being. These include:

  • eating a nutritious, well-balanced diet
  • exercising regularly
  • avoiding smoking
  • limiting alcohol consumption
  • seeking counseling for depression, anxiety, and other mental health difficulties

Ampullary cancer is a rare cancer. It affects the small opening called the ampulla of Vater, which is located on the duodenum. The duodenum is the first section of the small intestine.

A person with ampullary cancer will likely develop obstructive jaundice because the cancer blocks the bile ducts. This causes yellowing of the skin and eyes alongside pale, foul-smelling stool and dark urine.

A person may also experience other symptoms, such as fatigue, back pain, unexplained weight loss, and diarrhea.

The outlook is generally favorable for people with ampullary cancer, as long as they receive treatment at an early stage. The treatment will typically include surgery and chemotherapy.