Ampullary cancer, or ampullary carcinoma, is a cancer that forms in a body part called the ampulla of Vater. The ampulla of Vater is a small opening that enters into the first portion of the small intestine, known as the duodenum. The ampulla of Vater is the spot where the pancreatic and bile ducts release their secretions into the intestines.

Facts about ampullary cancer

Ampullary cancer is a fairly rare form of cancer. In fact, fewer than 1% of cancers related to the gastrointestinal tract are ampullary cancer.

Types of ampullary cancer

True ampullary cancers originate in the ampulla of Vater. They are often confused with periampullary cancers. These originate in the pancreas, bile duct, or intestines close to the ampulla of Vater. In general, cancers that are ampullary have a better survival rate than periampullary cancers.

Symptoms

Jaundice (yellowing of the skin and eyes) is the most common symptom of ampullary cancer. This is because the tumor in the ampulla of Vater blocks the bile duct. Instead of flowing into the intestines, the bile enters the bloodstream and causes yellowing of the skin. Other symptoms of ampullary cancer include:

• Loss of appetite and weight loss
• Abdominal (belly) pain
• Back pain
• Pruritus, or skin itchiness, associated with the jaundice
• Stomach upset and vomiting
• Diarrhea
• Gastrointestinal bleeding
• Pancreatitis (inflammation of the pancreas)
• Pale, greasy stools

Diagnosis

Because jaundice is the most common symptom of ampullary cancer, this is usually the physical symptom that will lead healthcare providers to look for ampullary cancer as a possibility. Tests that healthcare providers will do to determine if ampullary cancer is the cause may include blood tests and urine tests to look for markers of the cancer.

Healthcare providers may use specific imaging scans to look for the presence of a tumor inside the ampulla of Vater. These may be ultrasounds, or CT or MRI scans.

Healthcare providers may also use endoscopy, in which a long, thin scope with a tiny video camera on the end is threaded down the mouth, through the esophagus and stomach, and into the duodenum to look at the ampulla. They may also use it to take samples to look for cancer cells.

Other tests might be done to look at the pancreatic and bile ducts to see if they are blocked. This might be done with endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP). 

Treatment

If your healthcare providers determine that you have ampullary cancer, the standard approach is to remove the tumor from the ampulla of Vater. A complex operation called Whipple procedure (pancreaticoduodenectomy) is used. This procedure involves the removal of the tumor in the affected portion of the ampulla of Vater and the surrounding areas. This includes the head of the pancreas, duodenum, gallbladder, part of the common bile duct, nearby lymph nodes, and sometimes part of the stomach. 

In some centers, the procedure is performed using endoscopic instruments. Long, narrow tools and a tiny video camera are used to perform the procedure are inserted through small incisions (cuts) in the belly. This makes the procedure minimally invasive (there's not a lot of cutting and bleeding) and can shorten the recovery from the surgery. However, the most common way to perform the Whipple procedure is with an open belly. 

Some healthcare providers might also suggest further (adjuvant) treatment after surgery with chemotherapy (and possible radiation therapy), although not all healthcare providers agree with this.

For people who are not able to tolerate a Whipple procedure, a less complex operation or another procedure (such as using a laser to destroy the tumor) might be done, although it is not clear if these techniques can cure ampullary cancers. 

Ampullary cancer is a life-threatening diagnosis, but people treated using the Whipple procedure have shown a 5-year survival rate ranging from about 20% to as high as 75%, based on how far the tumor has progressed.

Prevention

Ampullary cancer is such a rare condition that experts aren’t sure what steps, if any, can be taken to prevent it.