What is Islet Cell Cancer

Islet cell cancer is a type of cancer that develops in the hormone-producing cells of the pancreas (islet cells). This type of cancer is also called pancreatic neuroendocrine cancer.

There are two main types of islet cell tumors:

• Tumors that create extra hormones (functional).
• Tumors that do not create extra hormones (non-functional).

What are the causes?

The cause of this condition is not known.

What increases the risk?

You are more likely to develop islet cell cancer if you have a syndrome called multiple endocrine neoplasia type 1 (MEN1). This syndrome is something that is passed down from a parent to a child (hereditary).

What are the signs or symptoms?

Symptoms of this condition vary depending on whether the tumor is functional or non-functional.

If the tumor is functional, symptoms can include:

• Diarrhea or loose stool.
• Digestive problems or nausea.
• Ulcers.
• Fatigue.
• Low blood sugar (hypoglycemia).
• High blood sugar (hyperglycemia).
• Unexplained weight gain or loss.
• Rash on the face, abdomen, or legs.

If a tumor is non-functional, there are usually no symptoms until the cancer has spread to other parts of the body. Symptoms can then include:

• A lump in the abdomen.
• Yellow skin and yellow in the whites of the eyes (jaundice).
• Indigestion.
• Diarrhea.
• Pain in the abdomen or back.

How is this diagnosed?

This condition may be diagnosed based on:

• Your symptoms, your medical history, and a physical exam.
• Tests, such as:
  • Blood tests.
  • CT scan of the abdomen.
  • MRI.
  • Ultrasound.
  • Endoscopic retrograde cholangiopancreatogram. This test examines the tubes (ducts) between the liver, gallbladder, and small intestine to see if they are blocked by a tumor.
  • Angiogram. This test examines the blood vessels and the flow of blood to see if the vessels are blocked by a tumor.
  • Biopsy. A small piece of tissue is removed from the pancreas or abdomen so it can be looked at under a microscope to check for signs of cancer.

If you have a family history of cancer, your health care provider may also test for inherited (genetic) syndromes that can increase your risk of developing cancer. You may also be referred to an expert who specializes in diagnosing and treating islet cell cancer.

How is this treated?

Treatment for this condition depends on the type of tumor, how much the cancer has spread in the body (what stage it is), and how likely it is to spread (what grade it is). Treatment can include:

• Surgery to help remove cancer from your body. The surgery will vary depending on the stage of the cancer. It may involve:
  • Removing the tumor (enucleation).
  • Removing parts of the pancreas, stomach, bile duct, and small intestine (Whipple procedure).
  • Removing part of the pancreas and the spleen (distal pancreatectomy).
  • Removing the spleen (splenectomy).
  • Removing the stomach (gastrectomy).
• Medicine to kill cancer cells or to slow their growth (chemotherapy).
• Medicine to attack a tumor’s genes and proteins (targeted therapy). These medicines attack the genes and proteins that allow a tumor to grow while limiting damage to healthy cells.
• Medicine to boost the body’s defense system to fight the tumor (immunotherapy).
• Medicine to remove or block hormones that allow the cancer to grow (hormone therapy).
• Medicine to block the flow of blood to the liver (hepatic arterial occlusion).

In some cases, a health care provider may delay treatment and closely monitor the tumor for growth (active surveillance).

Follow these instructions at home:

• Learn about your disease and any possible side effects from treatment. This will help you talk about your choices with your health care provider and decide on treatments that are right for you.
• Take over-the-counter and prescription medicines only as told by your health care provider.
• Return to your normal activities as told by your health care provider. Ask your health care provider what activities are safe for you.
• Eat a healthy diet. A healthy diet includes lots of fruits and vegetables, low-fat dairy products, lean meats, and fiber.
  • Make sure half your plate is filled with fruits or vegetables.
  • Choose high-fiber foods such as whole-grain breads and cereals.
• Drink plenty of water and decaffeinated beverages.
• Limit alcohol intake to no more than 1 drink a day for nonpregnant women and 2 drinks a day for men. One drink equals 12 oz of beer, 5 oz of wine, or 1½ oz of hard liquor.
• Get regular exercise. Aim for 30 minutes of moderate-intensity activity 5 times a week. Examples of moderate-intensity activity include walking and yoga. Be sure to talk with your health care provider before starting any exercise routine.
• Keep all follow-up visits as told by your health care provider. This is important.

Where to find more information

• National Cancer Institute: www.cancer.gov  
• American Cancer Society: www.cancer.org  

Contact a health care provider if:

• You have abdominal pain or nausea.
• You have diarrhea or a change in bowel movements.
• You have swelling or redness anywhere, especially around a cut or wound.
• You have a headache or sinus pain.
• You have skin sores or a rash.
• You have pain or burning when urinating.
• You have unexplained weight loss.

Get help right away if:

• You have a fever.
• You have chest pain or shortness of breath.
• You have blood in your urine.
• You have a severe headache with a stiff neck.
• You are confused.
• You have swelling, pain, or tenderness in your arms or legs.

Summary

• Islet cell cancer is a type of cancer in which tumors develop in the hormone-producing cells of the pancreas (islet cells). This disease is also called pancreatic endocrine cancer.
• You are more likely to develop islet cell cancer if you have a syndrome called multiple endocrine neoplasia type 1 (MEN1).
• Symptoms can include diarrhea, digestive problems, ulcers, low blood sugar (hypoglycemia), high blood sugar (hyperglycemia), unexplained weight gain or loss, or a rash on the face, abdomen, or legs.
• Treatment may include surgery, chemotherapy, targeted therapy, immunotherapy, or hormone therapy. In some cases, a combination of treatments is used.