Islet cell cancer, a rare cancer, is a disease in which malignant cells are found in certain tissues of the pancreas. The pancreas is about 6 inches long and is shaped like a thin pear, wider at one end and narrower at the other. The pancreas lies behind the stomach, inside a loop formed by part of the small intestine. The broader right end of the pancreas is called the head, the middle section is called the body, and the narrow left end is the tail.

The pancreas has two basic jobs in the body. It produces digestive juices that help break down (digest) food, and hormones (such as insulin) that regulate how the body stores and uses food. The area of the pancreas that produces digestive juices is called the exocrine pancreas. About 95% of pancreatic cancers begin in the exocrine pancreas. The hormone-producing area of the pancreas has special cells called islet cells and is called the endocrine pancreas. Only about 5% of pancreatic cancers start here.

The islet cells in the pancreas make many hormones, including insulin, which help the body store and use sugars. When islet cells in the pancreas become cancerous, they may make too many hormones. Islet cell cancers that make too many hormones are called functioning tumours. Other islet cell cancers may not make extra hormones and are called non-functioning tumours. Tumours that do not spread to other parts of the body can also be found in the islet cells.  A doctor will need to determine whether the tumour is cancer or a benign tumour.

A doctor should be seen if there is pain in the abdomen, diarrhoea, stomach pain, a tired feeling all the time, fainting, or weight gain without eating too much.

If there are symptoms, the doctor will order blood and urine tests to see whether the amounts of hormones in the body are normal. Other tests, including x-rays and special scans, may also be done.

The chance of recovery (prognosis) depends on the type of islet cell cancer the patient has, how far the cancer has spread, and the patient’s overall health.

Stages of islet cell cancer

Once islet cell cancer is found, more tests will be done to find out if cancer cells have spread to other parts of the body. This is called staging. The staging system for islet cell cancer is still being developed. These tumours are most often divided into one of three groups:

1. islet cell cancers occurring in one site within the pancreas,
2. islet cell cancers occurring in several sites within the pancreas, or
3. islet cell cancers that have spread to lymph nodes near the pancreas or to distant sites.

A doctor also needs to know the type of islet cell tumour to plan treatment. The following types of islet cell tumours are found:

Gastrinoma

The tumour makes large amounts of a hormone called gastrin, which causes too much acid to be made in the stomach. Ulcers may develop as a result of too much stomach acid.

Insulinoma

The tumour makes too much of the hormone insulin and causes the body to store sugar instead of burning the sugar for energy. This causes too little sugar in the blood, a condition called hypoglycaemia.

Glucagonoma

This tumour makes too much of the hormone glucagon and causes too much sugar in the blood, a condition called hyperglycaemia.

Miscellaneous

Other types of islet cell cancer can affect the pancreas and/or small intestine. Each type of tumour may affect different hormones in the body and cause different symptoms.

Recurrent

Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the pancreas or in another part of the body.

How islet cell cancer is treated

There are treatments for all patients with islet cell cancer. Three types of treatment are used:

• Surgery (taking out the cancer).
• Chemotherapy (using drugs to kill cancer cells).
• Hormone therapy (using hormones to stop cancer cells from growing).

Surgery is the most common treatment of islet cell cancer. The doctor may take out the cancer and most or part of the pancreas. Sometimes the stomach is taken out (gastrectomy) because of ulcers. Lymph nodes in the area may also be removed and looked at under a microscope to see if they contain cancer.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in the vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body.

Hormone therapy uses hormones to stop the cancer cells from growing or to relieve symptoms caused by the tumour.

Hepatic arterial occlusion or embolization uses drugs or other agents to reduce or block the flow of blood to the liver in order to kill cancer cells growing in the liver.

Gastrinoma

Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Surgery to remove the stomach (gastrectomy).
3. Surgery to cut the nerve that stimulates the pancreas.
4. Chemotherapy.
5. Hormone therapy.
6. Hepatic arterial occlusion or embolization to kill cancer cells growing in the liver.

Insulinoma

Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Chemotherapy.
3. Hormone therapy.
4. Drugs to relieve symptoms.
5. Hepatic arterial occlusion or embolization to kill cancer cells growing in the liver.

Glucagonoma

Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Chemotherapy.
3. Hormone therapy.
4. Hepatic arterial occlusion or embolization to kill cancer cells growing in the liver.

Miscellaneous Islet Cell Cancer

Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Chemotherapy.
3. Hormone therapy.
4. Hepatic arterial occlusion or embolization to kill cancer cells growing in the liver.

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