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Ampullary cancer

This page was reviewed under our medical and editorial policy by

Maurie Markman, MD, President, Medicine & Science at CTCA.

This page was reviewed on August 24, 2022.

Ampullary cancer is a type of cancer that develops in a small opening called the ampulla of Vater, where the pancreatic and bile ducts meet and then empty into the small intestine. It’s not common, accounting for around 7 percent of cancers that originate in the periampullary area of the body and less than 1 percent of cancers that originate in the gastrointestinal tract, according to the American Society of Clinical Oncology.

Generally, ampullary cancer is diagnosed in patients 70 and older, but it may also occur earlier in patients with pre-existing health conditions.

What is ampullary cancer?

Ampullary cancer originates in the ampulla of Vater, which was named after the scientist who first described it. An ampulla is an enlarged opening at the end of a duct or canal in the body, and the ampulla of Vater is responsible for allowing both the pancreatic and bile ducts to empty into the duodenum, or small intestine.

When ampullary cancer occurs, even if it’s small, it often blocks the bile duct. This may cause noticeable side effects, like yellowing of the skin, which may make it easier to notice that something’s wrong. The clear visibility of this symptom means ampullary cancer is often diagnosed earlier than many types of pancreatic cancer. Although ampullary cancer isn’t a type of pancreatic cancer, the two are sometimes grouped together due to the similarity of treatment options.

Causes of ampullary cancer

Medical professionals aren’t completely clear about what causes ampullary cancer because the tumors often occur with no precipitating medical issue. However, people with hereditary polyposis syndrome or nonpolyposis colorectal cancer (Lynch syndrome) may be at a higher risk for this type of cancer. Both of these rare inherited conditions are related, not only to the growth of polyps and colon cancer, but to other cancer risks as well.

Symptoms of ampullary cancer

Symptoms of ampullary cancer include:

  • Jaundice, or yellowing of the skin and eyes (most common symptom)
  • Weight loss
  • Fatigue
  • Gastrointestinal bleeding, including blood in stool
  • Diarrhea
  • Unexplained weight loss
  • Back pain

It’s important to note that the presence of one or more of these symptoms doesn’t necessarily mean they’re being caused by ampullary cancer. Patients should seek advice and speak to a doctor about any concerns involving changes to their body.

Diagnosis

Two common methods that doctors use to diagnose ampullary cancer are:

Endoscopic ultrasound: An ultrasound combined with X-rays allows a doctor to look for the presence of a tumor. If a tumor is found, the doctor will then perform a biopsy to examine the tissue sample under a microscope and look for the presence of cancer cells.

CT scan: This imaging tool takes clear pictures of the inside of the body. Doctors use this to stage the cancer to see whether it’s spread.

Ampullary cancer staging

Doctors use the TNM system to diagnose ampullary cancer. This system provides concrete knowledge about the cancer, such as the size of the tumor and whether it’s spread to any other parts of the body.

TNM staging stands for:

T (tumor): This describes the size of the original tumor.

N (node): This refers to whether cancer has been found in the lymph nodes.

M (metastasis): This refers to whether the cancer has spread to other parts of the body.

Together, the three elements of the TNM system make for an effective way to understand more about a patient’s cancer and to identify appropriate treatment options.

If ampullary cancer metastasizes, it tends to spread to the liver or abdominal lymph nodes. Generally, ampullary cancers are less aggressive than other cancer types that affect the head of the pancreas.

Treatment

The care team will recommend a treatment plan based on the type of cancer, personal preferences and any pre-existing health conditions. The most commonly used treatments include:

Surgery

Surgery is the only treatment option, used to remove the cancer. A surgical resection with pancreaticoduodenectomy, also known as a Whipple procedure, is a complex operation that removes part of the pancreas and small intestine.

Radiation therapy

For patients with a high risk of cancer recurrence, doctors may recommend radiation therapy after surgery. This treatment uses high-energy X-rays to specifically target and kill cancer cells.

Chemotherapy

For certain types of ampullary cancer, such as cases that have spread to the lymph nodes, chemotherapy may be used either after surgery or as a stand-alone treatment. Chemotherapy uses drugs, usually administered intravenously, to kill cancer cells.

Survival rate

Ampullary cancer has shown an average five-year survival rate of 30 to 67 percent after surgery, according to a study in Scientific Reports. However, rates vary from person to person and are affected by cancer subtypes, the overall health of the patient and how early the cancer was detected.

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