Ampullary (AM-poo-la-ree) cancer is a rare cancer that forms in an area of your digestive system called the ampulla of Vater. The ampulla of Vater is located where your bile duct and pancreatic duct join and empty into your small intestine.

Ampullary cancer forms near many other parts of the digestive system, such as the liver, pancreas and small intestine. When ampullary cancer grows, it may affect these other organs.

Ampullary cancer treatment often involves extensive surgery to remove the cancer and a large margin of healthy tissue.

Signs and symptoms of ampullary cancer may include:

• Yellowing of the skin and eyes (jaundice)
• Clay-colored stools
• Abdominal pain
• Fever
• Bleeding from the rectum
• Nausea
• Vomiting
• Weight loss

When to see a doctor

Make an appointment with your doctor if you have any persistent signs or symptoms that worry you.

It's not clear what causes ampullary cancer.

In general, cancer starts when cells develop changes (mutations) in their DNA. A cell's DNA contains the instructions that tell the cell what to do. The changes tell the cell to begin multiplying uncontrollably and to continue living when normal cells would die. The accumulating cells form a tumor that can invade and destroy normal body tissue.

Factors that can increase the risk of ampullary cancer include:

• Your age. Ampullary cancer is more common in adults older than 70.
• Being male. Males are slightly more likely to develop ampullary cancer than are females.
• Inherited syndromes that increase cancer risk. Some gene mutations passed through generations of your family can increase your risk of ampullary cancer significantly. Only a small percentage of ampullary cancers are linked to inherited genes. The most common inherited syndromes that increase ampullary cancer risk are familial adenomatous polyposis and Lynch syndrome, which is also known as hereditary nonpolyposis colorectal cancer.

Tests and procedures used to diagnose ampullary cancer include:

• Passing a thin, flexible scope down your throat (endoscopy). Endoscopy is a procedure to examine your digestive system with a long, thin tube (endoscope) equipped with a tiny camera. The endoscope is passed down your throat, through your stomach and into your small intestine to view the ampulla of Vater.

  Special surgical tools can be passed through the endoscope to collect a sample of suspicious tissue.

  Endoscopy can also be used to create images. For instance, endoscopic ultrasound may help capture images of ampullary cancer.

  Doctors may also inject a dye into your bile duct using endoscopy in a procedure called endoscopic retrograde cholangiopancreatography. The dye shows up on X-rays and can show blockages in your bile duct or pancreatic duct.

• Imaging tests. Imaging tests may help your doctor understand more about your cancer and determine whether it has spread beyond the ampulla of Vater. Imaging tests may include endoscopic ultrasound, endoscopic retrograde cholangiopancreatography, magnetic resonance cholangiopancreatography and computerized tomography.
• Testing cancer cells in the laboratory. A sample of cancer cells removed during endoscopy or surgery can be analyzed in the lab to look for characteristics that might guide treatment and prognosis.

Ampullary cancer treatment options may include:

• Surgery to remove the pancreas and small intestine. The Whipple procedure (pancreaticoduodenectomy) involves removing the head of your pancreas as well as a portion of your small intestine (duodenum), your gallbladder and part of your bile duct.

  The Whipple procedure can be done using a large incision in your abdomen, or as a minimally invasive surgery, which uses several small incisions.

• Minimally invasive surgery. For very small ampullary cancers and precancerous tumors, it may be possible to remove the cancer with tools passed through an endoscope (endoscopic surgery). This may be an option in certain situations.

• Combined chemotherapy and radiation. Chemotherapy uses drugs to kill cancer cells. Radiation therapy uses beams of energy, such as X-rays and protons, to kill cancer cells. Used together, these treatments may be more effective for ampullary cancers.

  Combined chemotherapy and radiation may be used before surgery, to make it more likely that a cancer can be removed completely during an operation. The combined treatment can also be used after surgery to kill any cancer cells that might remain.

• Chemotherapy alone. Chemotherapy is sometimes used after surgery to kill cancer cells that might remain. In people with advanced ampullary cancer, chemotherapy may be used alone to slow the growth of the cancer.
• Treatment to reduce pain and discomfort. If other treatments aren't possible or aren't helping, your doctor may recommend treatments that focus on making you feel more comfortable. This can include surgery to place a small wire mesh tube (stent) in your bile ducts to drain fluid that causes your skin and eyes to yellow.

A cancer diagnosis can permanently change your life. Each person finds his or her own way of coping with the emotional and physical changes cancer brings. But when you're first diagnosed with cancer, sometimes it's difficult to know what to do next.

Here are some ideas to help you cope:

• Learn enough about cancer to make decisions about your care. Ask your doctor about your cancer, including your treatment options and, if you like, your prognosis. As you learn more about cancer, you may become more confident in making treatment decisions.
• Keep friends and family close. Keeping your close relationships strong will help you deal with your cancer. Friends and family can provide the practical support you'll need, such as helping take care of your home if you're in the hospital. And they can provide emotional support when you feel overwhelmed by cancer.

• Find someone to talk with. Find a good listener who is willing to hear you talk about your hopes and fears. This may be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group also may be helpful.

  Ask your doctor about support groups in your area. Other sources of information include the National Cancer Institute and the American Cancer Society.

Start by making an appointment with your family doctor if you have any signs or symptoms that worry you. If your doctor suspects you might have ampullary cancer, you might be referred to a doctor who specializes in treating diseases and conditions of the digestive system (gastroenterologist) or a doctor who specializes in treating cancer (oncologist).

Because appointments can be brief, and because there's often a lot of information to cover, it's a good idea to be well prepared. Here's some information to help you get ready, and what to expect from your doctor.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes.
• Make a list of all medications, vitamins or supplements that you're taking.
• Consider taking a family member or friend along. Sometimes it can be difficult to take in all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions will help make the most of your time together. List questions from most important to least important in case time runs out. For ampullary cancer, some basic questions to ask your doctor include:

• Can you explain what my test results mean?
• Do you recommend any other tests or procedures?
• What is the stage of my ampullary cancer?
• What are my treatment options?
• What side effects are likely with each treatment?
• How will treatment affect my daily life?
• Which treatment options do you think are best for me?
• How likely is it that I'll achieve remission with the treatments you recommend?
• How quickly must I make a decision on my treatment?
• Should I get a second opinion from a specialist? What will that cost, and will my insurance cover it?
• Are there brochures or other printed material that I can take with me? What websites do you recommend?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions that occur to you.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may allow time later to cover other points you want to address. Your doctor may ask:

• When did you first begin experiencing symptoms?
• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?