Ampullary Cancer: What You Should Know About This Rare GI Cancer

Causes and risk factors for ampullary cancer

Doctors believe most ampullary cancers arise spontaneously — they aren’t usually due to lifestyle factors, family history, or other possible risks. Doctors most commonly diagnose ampullary cancer in people who are in their 70s.

However, doctors have found some connections with certain medical conditions. These conditions are inherited and may increase your risk of ampullary cancer. They include hereditary polyposis syndrome and hereditary nonpolyposis colorectal cancer.

Hereditary polyposis syndrome increases the likelihood a person will develop polyps in their digestive tract. The presence of polyps can increase a person’s risk of colorectal cancer.

Hereditary nonpolyposis colorectal cancer increases a person’s risk of developing colorectal cancer where polyps are either very small or not present at all.

Having either of these conditions can increase your risk of ampullary cancer 200 percent.

Common symptoms of ampullary cancer

To understand ampullary cancer’s symptoms, it’s important to know more about its location in the body.

• The gallbladder is an organ responsible for secreting bile, a substance that helps the body digest fats.
• The bile secreted by the gallbladder travels down the common bile duct, which is sort of like a pathway. From there, it empties into the small intestine.
• The pancreas is an organ that, among other tasks, releases a substance called pancreatic juice. Pancreatic juice helps to ensure digestion.
• Pancreatic juice travels down the pancreatic duct to empty into the small intestine.
• The area where the pancreatic duct and the bile duct meet is called the ampulla of Vater. This is where ampullary cancer occurs.

Ampullary cancer can affect the functions of the organs around the ampulla of Vater. This includes the liver, pancreas, and small intestine. Examples of symptoms include:

• diarrhea
• fatigue
• gastrointestinal bleeding, which usually appears as dark, tarry, or bloody stools
• pain that radiates to the back
• weight loss

The most common symptom in those with ampullary cancer is obstructive jaundice. This occurs when bile can’t pass to the small intestine. Symptoms associated with obstructive jaundice include:

• abdominal pain
• dark urine
• fatigue
• fever
• itchy skin
• mental status changes, such as confusion
• pale-colored stool
• stools that are especially foul-smelling
• yellowing of the eyes or skin

If you experience these symptoms, your doctor will typically begin testing for underlying causes, which could include ampullary cancer.

Recurrence symptoms for ampullary cancer

Recurrence is when a cancer comes back after treatment. When ampullary cancer recurs, the cancer doesn’t always occur in the ampulla of Vater. Instead, 2022 research shows that cancerous cells may grow in areas such as the liver, lining of the abdominal cavity, lungs, or in multiple locations. The most common recurrence area is the liver.

As a result, recurrence symptoms can be different. Liver cancer symptoms may include unexplained weight loss, appetite loss, and feeling very full, even after just eating a small amount. If you have symptoms and are uncertain if they could be related to a history of ampullary cancer, talk with your doctor.

Stages of ampullary cancer

A doctor will use imaging studies, and possibly biopsies of the tumor, to determine what stage your ampullary cancer is. The most common staging system is the TNM system. This stands for tumor, nodes, and metastases.

The tumor (T) staging describes where the initial tumor has spread in the body. The categories include:

• T1a. The tumor is limited to the sphincter of Oddi.
• T1b. The tumor has invaded the duodenal submucosa.
• T2. The tumor has invaded the duodenal proper muscle.
• T3a. The tumor has invaded the pancreas less than or equal to 0.5 centimeters.
• T3b. The tumor has invaded the pancreas greater than 0.5 centimeters.
• T4. The tumor involves the celiac axis or superior mesenteric artery.

Nodes (N) refers to if the cancer has spread to nearby lymph nodes. This includes:

• N0. No nodal metastasis has occurred.
• N1. Between 1 and 3 nodal metastases have occurred.
• N2. Greater than 4 nodal metastases have occurred.

Metastases (M) refers to metastasis, or if the cancer has spread to nearby organs. The more a cancer has spread, the more difficult the cancer usually is to treat.

• M0. There is no distant metastasis.
• M1. Distant metastasis has occurred.

Treatment options for ampullary cancer

Ampullary cancer treatment options depend on what stage the cancer is in. Because this cancer type is so rare, a 2020 review found that not much information is available to doctors about the effectiveness of different treatments.

Most treatments involve a combination of surgery and chemotherapy. Doctors may also recommend radiation to shrink or destroy a tumor, but this treatment is less common.

Early stage disease treatments

In early stage disease where the cancer hasn’t spread, a doctor will usually recommend surgery known as a pancreaticoduodenectomy. Another name for this procedure is a Whipple. In this procedure, a surgeon removes the:

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

Following the surgery, a doctor will usually recommend taking a medication to kill any remaining cancer cells. This is known as chemotherapy. The medication for ampullary cancer after a Whipple procedure is usually gemcitabine (Gemza), a medication that keeps cancer cells from rapidly dividing.

Treatments for late stage disease or unresectable tumor

Sometimes, ampullary cancer has spread to other organs or its location is unresectable, meaning a surgeon can’t remove it. When this happens, a doctor may prescribe chemotherapy medications to keep the cancer from spreading further. Ideally, this will also reduce symptoms.

A doctor may prescribe a combination of chemotherapy medications, such as an antimetabolite, like fluoropyrimidine or gemcitabine, with a platinum compound, like cisplatin or oxaliplatin. These medications attack cancer cells in different ways to keep the cancer from spreading.

Prognosis and outlook for ampullary cancer

Ampullary cancer tends to cause symptoms at earlier stages than other cancer types, such as pancreatic cancer. This helps to improve outcomes because the cancer is diagnosed at earlier, more treatable stages.

The 5-year survival rate for those with ampullary cancer whose cancer has not spread to nearby lymph nodes is 70 to 80 percent. The 5-year survival rate for cancer that has spread is 20 to 50 percent.

An estimated 45 percent of people treated with surgery for ampullary cancer experience a recurrence of their cancer. If a person has a tumor at stage T2 or higher, a doctor will usually recommend chemotherapy to prevent cancer recurrence.

As a note, these numbers are based on studies that (while still recent) are a couple of years old. Cancer research is constantly improving and the exact statistics of your prognosis are likely to be more positive.

Takeaway

Ampullary cancer is a rare gastrointestinal cancer that has a good outlook with early treatment but is known to recur.

Although the condition most commonly occurs with no family history, those with certain hereditary conditions should talk with their doctor about their need for additional screening.