What is periampullary cancer?

Periampullary cancer is a rare type of cancer that forms in an area of the head of the pancreas, the distal common bile duct and the first part of the small intestine, close to the ampulla of Vater. Ampullary cancers form inside the ampulla of Vater itself – an enlargement of the ducts from the liver and pancreas where they join and enter the small intestine. As these types of tumour grow, they may affect the surrounding organs, such as the liver, pancreas and small intestine. Some periampullary cancers may spread (metastasise) to the liver, lymph nodes, lungs, peritoneum (abdominal lining) and other organs.

What are the symptoms of periampullary cancer?

Signs and symptoms of periampullary cancer may include: ● Yellowing of the skin and white of eyes (jaundice) ● Clay-coloured stools ● Abdominal pain ● Fever ● Bleeding from the rectum ● Nausea ● Vomiting ● Unintended weight loss Jaundice occurs when a tumour blocks the bile duct, leading to a build-up of bile fluid. Signs of jaundice often lead to these cancers being detected earlier than most pancreatic cancers, which can improve prognosis. These symptoms may also be caused by other medical problems. Consult your doctor if you have persistent signs or symptoms that worry you.

What causes periampullary cancer?

The cause of periampullary cancer is not clear and research is ongoing. Some factors which appear to increase the risk of periampullary cancer have been identified, including: ● Being over the age of 70 years ● Men are slightly more affected by periampullary cancer than women ● Family history of rare genetic syndromes that can increase cancer risk.

How is periampullary cancer diagnosed?

A combination of diagnostic tests may be required, such as: ● Imaging tests, such MRI, CT scan or ultrasound ● Blood or urine tests to check for cancer markers ● Endoscopy – passing a thin, flexible tube with a tiny camera (endoscope) down the oesophagus, through the stomach into the small intestine ● Biopsy – collecting a small tissue sample to be analysed in the laboratory – can be done during endoscopy ● Endoscopic Ultrasound (EUS) – using an endoscope and ultrasound device to make images of the pancreas, bile ducts and small intestine ● Endoscopic retrograde cholangiopancreatography (ERCP) – using an endoscopic ultrasound device and dye injected into the pancreatic duct and /or bile duct, to look for blockages.

How is periampullary cancer treated?

Surgical procedures Surgical removal, along with a large margin of healthy tissue, is often the most successful treatment for periampullary cancer. Minimally invasive (keyhole) surgery, using an endoscope is an option in some cases. The Whipple procedure is commonly used. This involves removing the head of the pancreas, the first part of the small intestine (duodenum), the gallbladder, part of the bile duct and nearby lymph nodes. The remaining parts of the pancreas, stomach and intestines are reconnected. When surgical removal is not possible, a small wire mesh tube (stent) can be placed in the bile ducts to drain fluid that causes jaundice.

Chemotherapy and radiation therapy Chemotherapy drugs (either injected or taken by mouth) may be used for inoperable cancers, to help kill cancer cells, relieve symptoms and prolong survival. It may also be used to kill any cancer cells remaining after surgery. Chemoradiation – a combination of chemotherapy and radiotherapy (using high-energy beams to destroy cancer cells) may be used before surgery to help shrink a tumour, or after surgery to reduce the risk of cancer recurring.

Palliative care For advanced periampullary cancer that is not amenable to surgery, chemotherapy or radiotherapy, treatment may focus on symptom relief and improving quality of life. Palliative care may also be provided to complement other treatments.