What Is Ampullary Cancer?

Ampullary cancer is an extremely rare type of cancer. It begins as a malignant tumor in a part of the body called the ampulla of Vater, which is located where the bile duct meets the small intestine. According to a 2014 study, ampullary cancers account for only 0.2% of cancers of the gastrointestinal (digestive) system. It is most often treated by surgery.

What Is the Ampulla of Vater?

To get a good idea of what and where the ampulla of Vater is, it's important to understand some related anatomical and physiological terms, such as:

• Duodenum: The first segment of the small intestine, where the initial process of digestion occurs
• Pancreas: A large glandular organ that secretes digestive juices (containing pancreatic enzymes) into the duodenum
• Common bile duct: A tubular structure that is part of the biliary tree, it is responsible for transporting bile from the liver and the gallbladder, through the pancreas and into the duodenum.
• Gallbladder: A pear-shaped organ that functions to store bile (which is produced in the liver) and releasing the bile into the duodenum to break down fats.
• Liver: A very large glandular organ that functions in the biliary system to produce bile.
• Biliary tree: A system of tubular structures that function to transport secretions from the liver, gallbladder, and pancreas, through a series of ducts, into the duodenum. The biliary tree is joined by the main pancreatic duct, forming the ampulla of Vater.
• Biliary: Referring to the pancreas and bile duct systems together, functioning to produce pancreatic juice and bile which assist in the breakdown of foods so they can be easily absorbed through the process of digestion.
• Bile: A greenish-brown fluid that is made in the liver and stored in the gallbladder: bile is transported to the duodenum to breakdown ingested fats for absorption.
• Pancreatic secretions: Contain pancreatic enzymes and other substances that help to breakdown ingested food for absorption in the duodenum.

The ampulla of Vater is a small projection with an opening through which bile and pancreatic juices flow into the small intestine (duodenum). The duodenum is where pancreatic enzymes and bile help to break down ingested food for absorption of nutrients.

Other names for the ampulla of Vater include:

• The hepatopancreatic duct
• The hepatopancreatic ampulla

Sometimes ampullary cancer is referred to as ampulla of Vater cancer.

When a tumor blocks the passageway of pancreatic secretions (into the duodenum), it interferes with both the biliary and pancreatic secretions. When this passageway gets blocked, a person will have a buildup of bile in the bloodstream, resulting in jaundice (a yellowing of the skin).

It’s important to note that ampullary cancer is not considered a type of pancreatic cancer, although the ampulla of Vater is a structure that butts up to the pancreas.

Anatomical Features

The ampulla of Vater is located at the junction where the common bile duct and the pancreatic duct join. The common bile duct is a tube that carries bile (a substance that helps to break down fats in the digestive system) from the liver and the gallbladder through the pancreas and into the duodenum.

The precise location ampulla of Vater—where ampullary cancer develops—is at the last segment of the common bile duct, as it passes through the small intestine. All bile and pancreatic secretions go through the ampulla of Vater before they enter the small intestine (duodenum).

This link between structures is the reason that ampullary cancer has a likelihood of spreading to various other regions (such as the duodenum, common bile duct, and pancreas).

Types of Ampullary Cancer

Ampullary cancer begins in the ampulla of Vater, but it is often confused with another type of cancer, called periampullary cancer. Periampullary cancers originate in the pancreas, bile duct, or the duodenum (all structures that are in close proximity to the ampulla of Vater).

Overall, cancers that are true ampullary cancers are known to have a better survival rate than periampullary cancers, according to Johns Hopkins Medical Center.

The most common type of ampullary carcinomas are considered adenocarcinomas. Carcinomas are the most common types of cancer. The arise from a specific type of tissue that lines internal organs (such as in the liver, kidneys, or the ampulla of Vater).

Carcinomas may be confined to their primary location, or they may spread to other parts of the body. When ampullary cancer metastasizes (spreads) it usually involves regional lymph nodes, liver, adjacent organs, and lungs.

Adenocarcinomas are a type of cancer that originates in glandular cells found in tissue that lines some internal organs and releases substances (such as those found in the pancreas that release pancreatic secretions).

Other sub-types of ampullary carcinomas include:

• Papillary
• Adenosquamous
• Mucinous

Ampullary Cancer Symptoms

The most common symptom of ampullary cancer is jaundice (yellowing of the skin and eyes). This occurs because the tumor, located in the ampulla of Vater, blocks the bile duct. Instead of bile being able to freely flow into the small intestine (the duodenum), it accumulates, entering the bloodstream where it causes jaundice.

The good news about this early symptom of ampullary cancer is that it helps the diagnostician to make an early diagnosis. In fact, jaundice is usually the first sign that a person has ampullary cancer. This results in better outcomes for ampullary cancer (compared to other types of gastrointestinal cancer and pancreatic cancer).

Other symptoms of ampullary cancer include:

• Loss of appetite
• Weight loss
• Pain in the abdomen and back
• Itchiness of the skin (called pruritus)
• Nausea and vomiting
• Diarrhea
• Bleeding of the gastrointestinal system
• Pancreatitis (inflammation of the pancreas)
• Stools that are pale in color and appear greasy (called steatorrhea)

Causes 

Although the cause of ampullary cancer is not well known, there are some common risks that are thought to be associated with a higher incidence of getting this type of rare cancer. For example, those with an inherited condition that causes polyps (growths) to form in the digestive tract may be at higher risk for getting ampullary cancer than those who don’t have polyps.

How Does Ampullary Cancer Develop?

Ampullary cancer occurs when the cells (located in the ampulla of Vater) begin to grow out of control; the abnormal growing cells may form a mass (called a tumor). If the tumor is left undetected and untreated for long enough, it can invade nearby regions, or even spread to distant areas of the body (metastasis).

Associated Conditions

A common genetic condition thought to be associated with ampullary cancer is called classic familial adenomatous polyposis (FAP) or classic FAP. It is diagnosed when a person develops more than 100 adenomatous colon polyps. This is a hereditary condition that causes the development of polyps in the digestive tract.

A polyp is noncancerous or benign tissue that forms a lump. People with FAP are thought to have an increased risk of developing cancer in the digestive organs (inlcuding the stomach, duodenum, pancreas, and the biliary tree).

The biliary tree includes the ampulla of Vater. Tumors of the ampulla of Vater may occur when the benign tissue in the polyp changes and becomes cancerous when it is not removed promptly.

Invasion and Metastases of Ampullary Cancer

Although ampullary cancer can spread to other parts of the digestive system, such as the pancreas, liver, and duodenum, it is still considered ampullary cancer that has invaded or metastasized to other organs, not pancreatic, liver, or duodenum cancer.

However, because of the risk of these other adjoining organs being involved, that is the reason that a Whipple procedure is the most common treatment for ampullary cancer.

Invasion is when a tumor grows so large that it extends into or “invades” nearby tissues. Metastasis describes the ability of cancerous cells to spread into the lymphatic system via local lymph nodes, and through the blood vessels, circulating into normal tissues, elsewhere, sometimes to distant locations in the body.

Risk Factors

When it comes to cancer, a risk factor involves anything that could increase the probability that a person will get a specific form of cancer. Risk factors differ from one form to another.

There are some risk factors—called modifiable risk factors—that can be changed. These are things like diet, lifestyle, or exposure to substances known to be carcinogens (cancer-causing agents). On the other hand, non-modifiable risk factors are things like a person's genetics, sex, or age.

For ampullary cancer, there are no proven risk factors, but several factors are thought to increase the risk of ampullary cancer, including:

• An inherited condition (such as FAP) that is known to cause polyps in the digestive tract
• Age (age increases the risk of most types of cancer)
• Inflammatory bowel disease (IBD)

Treatment

Surgery is the primary treatment modality recommended for ampullary cancer. The most common surgical procedure performed for this type of cancer is called a Whipple procedure.

A Whipple procedure, also called a pancreaticoduodenectomy, involves removal of the tumor in the ampulla of Vater, as well as the removal of several adjoining areas that have a risk of developing cancer, these include:

• Removal of the head of the pancreas
• Removal of part of the duodenum
• Removal of the gallbladder (cholecystectomy)
• Removal of nearby lymph nodes
• Removal of part of the stomach (in some Whipple procedures, but not all)

A Whipple procedure is a major procedure that involves several procedures in one operation. Oftentimes, chemotherapy (medication that treats cancer) is ordered after recovery from a Whipple procedure.

Other Types of Treatment

Some people are not healthy enough to endure a Whipple procedure (because of their age or general health). Other treatment modalities that may be ordered instead of a Whipple procedure include:

• Laser treatment
• Chemotherapy
• Radiation therapy  

Treatment Team

When a procedure such as a Whipple procedure is performed, or other treatment is necessary for ampullary cancer, it’s important to have a team of healthcare providers experienced in treating ampullary and related types of cancer.

In fact, many experts recommend that a person undergoing a procedure such as a Whipple procedure, should only be operated on by an expert who has a proven track record in performing pancreatic surgery.

Coordinated care from doctors and other professionals on your care team may include:

• A gastroenterologist (a physician specializing in conditions of the gastrointestinal [digestive] tract)
• An oncologist (a cancer specialist)
• Radiation oncologists (specializing in treating cancer with radiation therapy)
• A surgeon who is experienced in performing pancreatic surgery
• Pathologists
• Social workers (to help with discharge planning and to help you access services needed at home such as home health care)
• Cancer support counselors
• Oncology nurses
• A dietician (to help instruct patients regarding diet restrictions after surgery)

Health Insurance and Advocacy

If you have been diagnosed with ampullary cancer, you may be interested in learning about the Affordable Care Act (ACA) law that ensures coverage for essential health benefits for cancer treatment and follow up care.

The ACA’s important features related to cancer treatment include:

• Health plans must cover essential cancer treatments (such as many types of cancer drugs) and follow up care.
• Health plans must cover checkup and cancer screenings such as mammograms and colonoscopies with no co-payment or deductible costs.
• Health insurance must be available to people with pre-existing conditions (including cancer).

A Word From Verywell

Having a diagnoses such as ampullary cancer, and preparing to begin treatment, is no small undertaking. If you haven’t done so already, it’s important to start taking care of yourself in ways you may not have in the past.

For example, finding a support group of other people going through similar circumstances can be a lifesaver when it comes to coping with the emotional aspects of having cancer. If you aren’t able to attend a group in person, there are many online resources. The American Cancer Society offers a list of resources available nationwide.

As you go through the daily challenge of coping with cancer, try to remember to stay in the moment, deal with today, then take on tomorrow when it comes. Don't be hesitate to reach out and ask for help or support when you need it, and be sure to engage in as many enjoyable and relaxing activities as possible. Do what you can to stay healthy and active during and after cancer treatment.

Last, but not least, try not to get stuck attaching to the label of being a cancer patient; keep in mind that many people have learned how to continue to maintain a high level of quality of life, even while on the cancer survivor path.