Ampullary Cancer

Ampullary cancer is a carcinoma that develops in the gastrointestinal tract. More specifically, it arises from the ampulla of Vater, which is a small opening where the bile and pancreatic ducts empty into the small intestine.

Ampullary cancer is a type of periampullary cancer. This is defined as a group of tumours that grow in close proximity to the ampulla of Vater in areas such as the pancreas, duodenum (the first segment of the small intestine) and the bile duct. However, unlike other periampullary cancers, symptoms of ampullary cancer tend to occur earlier in the disease and has a better prognosis. It is also difficult to distinguish from other periampullary cancer preoperatively.

While it is most common in adults over 70 years old and is more prevalent in men, people of any gender can develop this disease at any age.

Treatment

If ampullary cancer is detected, it will be staged and graded based on size, metastasis, and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.

Cancers can be staged using the TNM staging system:

  • T (tumour) indicates the size and depth of the tumour.
  • N (node) indicates whether the cancer has spread to nearby lymph nodes.
  • M (metastasis) indicates whether the cancer has spread to other parts of the body.

This system can also be used in combination with a numerical value, from stage 0 – IV:

  • Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
  • Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and has not spread to lymph nodes. This stage is also known as early-stage cancer.
  • Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
  • Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also classified as localised cancer.
  • Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.

Cancers can also be graded based on the rate of growth and how likely they are to spread:

  • Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
  • Grade II: cancer cells present as abnormal and grow faster than grade I cancers. This is also known as an intermediate-grade tumour.
  • Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.

Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate course of treatment for you.

Treatment is dependent on several factors, including location, stage of disease and overall health. There is no one treatment option for this type of cancer, due to the rarity of the disease.

Treatment options for ampullary cancer may include:

  • Surgery, potentially including pancreaticoduodenectomy or Whipple procedure.
  • Radiation therapy.
  • Chemotherapy.
  • Therapeutic embolisation.
  • Clinical trials.
  • Palliative care.

Risk factors

Because of how rare ampullary cancer is, there are few identifiable risk factors for this disease. The risk factors that have been determined for this disease are:

  • Being over 70 years of age.
  • Being male.
  • Inherited gastrointestinal conditions (such as familial adenomatous polyposis and hereditary nonpolyposis colorectal cancer (Lynch syndrome)).

Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.

Symptoms

The symptoms of ampullary cancer will vary depending on stage and whether the tumour has metastasised (spread to other parts of the body). Some of these symptoms include:

  • Jaundice.
  • Unexplained weight loss/loss of appetite.
  • Abdominal and/or back pain.
  • Pale, clay-coloured stools.
  • Rectal bleeding.
  • Fever.
  • Nausea and/or vomiting.
  • Pruritus.
  • Diarrhoea.
  • Pancreatitis.

Not everyone with the symptoms above will have cancer but see your GP if you are concerned.

Diagnosis

If your doctor suspects you have ampullary cancer, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment:

  • Physical examination.
  • Imaging tests, potentially including:
    • Ultrasound.
    • MRI (magnetic resonance imaging).
    • CT (computed tomography) scan.
    • Endoscopic ultrasound.
  • Blood tests.
  • Endoscopy.
  • Biopsy.

References

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