Ampullary cancer, or ampullary carcinoma, is a life-threatening 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.
Ampullary cancer is a fairly rare form of cancer. In fact, fewer than one percent of cancers related to the gastrointestinal tract are ampullary cancer.
True ampullary cancers originate in the ampulla of Vater. They are often confused with periampullary cancers, which 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.
Jaundice 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:
Progressive weight loss
Abdominal (belly) pain
Pruritus, or skin itchiness, associated with the jaundice
Stomach upset and vomiting
Loss of appetite
Pancreatitis (inflammation of the pancreas)
Pale, greasy stools
Because jaundice is the most common symptom of ampullary cancer, this is the physical symptom that will lead doctors to look for ampullary cancer as a possibility. Tests that doctors will do to determine if ampullary cancer is the cause may include blood tests and urine tests to look for markers of the cancer.
Doctors may use specific scans to look for the presence of a tumor inside the ampulla of Vater. These may be ultrasounds, or CT or MRI scans.
Doctors 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 and perhaps take samples to look for cancer cells.
If your doctors determine that you have ampullary cancer, the standard approach is to remove the tumor from the ampulla of Vater with a procedure called pancreaticoduodenal resection, or Whipple procedure. This procedure involves the resection, or removal, of the tumor in the affected portion of the ampulla of Vater and the surrounding areas.
The procedure is performed using endoscopic instruments. That means that the long, narrow cameras and tools used to perform the procedure are inserted through small incisions (cuts) in the abdomen. This makes the procedure minimally invasive (there's not a lot of cutting and bleeding) and improves the chances of recovering from the surgery.
Ampullary cancer is a life-threatening diagnosis, but thanks to the use of the Whipple procedure, the survival rates from the cancer are improving. In the last decade, people treated using the Whipple procedure have shown a five-year survival rate ranging from about 20 percent to as high as 75 percent, based on how far the tumor has progressed.
Ampullary cancer is such a rare condition that experts aren’t sure what steps, if any, can be taken to prevent it. It does appear to be more common among men than women.