Ampullary cancer: symptoms, diagnosis and treatment in the UK

The ampulla, also called the ampulla of Vater, is part of the digestive system. Cancer Research UK explains that when you eat, the gallbladder releases bile into the common bile duct, which joins the pancreatic duct. The bile and pancreatic juice then flow through the ampulla into the duodenum, the first part of the small bowel, where they help break down food.

Ampullary cancer is when abnormal cells in the ampulla divide in an uncontrolled way. Most start in the epithelial cells lining the ampulla and are called adenocarcinomas, and the cells can resemble those lining the duodenum, the pancreas and common bile duct, or a mix. Knowing which type of cell the cancer started in may help decide the best treatment.

Cancer Research UK notes that very rarely cancer can start in neuroendocrine cells in the ampulla, and that this is different from ampullary cancer.

Cancer Research UK says doctors do not know what causes ampullary cancer but think there may be a link with some health conditions caused by inherited gene changes, including familial adenomatous polyposis (FAP). There may also be a link with Lynch syndrome, but the evidence is less clear.

Cancer Research UK lists jaundice, blood in the poo, blood in the sick and inflammation of the pancreas (pancreatitis) as possible symptoms, and stresses that these symptoms can be caused by many other conditions that are much more common than ampullary cancer.

Cancer Research UK says to see your GP urgently or call NHS 111 if you have symptoms of pancreatitis or jaundice, or if you had blood in your poo or sick but it has stopped and you have no other symptoms.

It advises going to A&E or calling 999 if you have bleeding from your bottom that is not stopping or there is lots of blood, or if you have blood in your sick and black poo or feel unwell.

The NHS jaundice page says yellow skin or eyes can be a sign of something serious such as liver disease, so you need urgent medical help, and that you may also have itchy skin, darker pee and paler poo than usual. It lists alcohol-related liver disease, gallstones, hepatitis, pancreatitis and sickle cell disease among the many possible causes, which is why jaundice always needs assessment and does not by itself mean cancer.

You should see a doctor if you have symptoms that could be due to ampullary cancer. They will examine you and you normally have blood tests to check your general health and how well the liver is working. You may be referred to a specialist for further tests.

Tests listed by Cancer Research UK include an ultrasound of the abdomen, a CT scan of the chest, abdomen and pelvis, an endoscopy (gastroscopy), an endoscopic ultrasound, and ERCP to look inside the pancreas and bile ducts. If an abnormal area is found at endoscopy a biopsy is taken and examined by a pathologist.

Cancer Research UK says treatment depends on the size and stage of the cancer, whether surgery is possible, any gene changes and your general health, and that the most common treatments are surgery and chemotherapy.

You usually have surgery if the cancer has not spread and you are well enough, because it is a major operation. A pylorus preserving pancreaticoduodenectomy (PPPD) removes part of the bile duct, pancreas and duodenum, plus the gallbladder and nearby lymph nodes. A Whipple's procedure also removes the lower part of the stomach. Very early stage cancers may be treated with a smaller operation, an ampullectomy, to remove the ampulla only.

After part of the pancreas is removed, the remaining part should be able to make enough insulin, though blood sugar is monitored, and you may need an enzyme supplement to digest food, with a dietitian supporting you. Chemotherapy may follow surgery to lower the chance of the cancer coming back, or may be used to control cancer that has spread, cannot be removed or has come back, aiming to control it and reduce symptoms.

Rarely, the cancer has gene changes. Cancer Research UK says the immunotherapy drug pembrolizumab may be used if it has microsatellite instability (MSI-high) or mismatch repair deficiency, and targeted drugs may be used for other changes where a drug exists.

Cancer Research UK says that after treatment you have regular follow-up appointments, with a CT scan and blood tests every 6 months to check for side effects and signs of the cancer coming back, usually for a number of years. If you have advanced cancer you may also be referred to a symptom control or palliative care team.

It acknowledges that coping with a rare cancer can be very difficult and that talking to your specialist nurse may help, as they can discuss support services and refer you to specialist teams.

On the NHS, a doctor can refer you to a specialist for tests, and Cancer Research UK says your specialist nurse can answer worries and help arrange support.

Private consultations and scans are available. Cancer Research UK describes surgery to remove ampullary cancer as a major operation, so it is reasonable to ask any provider about the surgeon's experience and who will manage your care afterwards.