Islet Cell Cancer

What is Islet Cell Cancer?

Islet Cell Cancer is an umbrella term used to describe a subset of cancers. These cancers are located in the pancreas, and attach themselves to hormone producing cells that produce insulin and glucogon. These cancers are also referred to as pancreatic neuroendocrine tumors and they are extremely rare.

The islet cells referred to in the term are responsible for producing hormones inside the pancreas. Some of the affected cells may still produce hormones, but generally produce more of the hormone than the body can use.

If the tumor is malignant, it can easily spread throughout the body via the bloodstream. Pancreatic cancer is considered to be extremely dangerous, and the prognosis is quite grim from the day of diagnosis. It is very important that patients follow their doctor’s directions, and their treatment plan carefully.

Pancreatic islet cell tumors are also referred to by other names by doctors. These names are:

  • Islet of Langerhans tumor
  • Pancreatic endocrine tumor
  • Neuroendocrine tumor

Islet cell cancerous tumors can be classified into two main categories, functional or nonfunctional. The subtypes are further broken down into the following tumor types:

  • Nonfunctional Islet Cell Tumors – These tumors create substances, but do not produce any symptoms. Usually, the only symptoms produced are due to the spread of the tumor cells. The majority of these tumors are malignant.
  • Functional Islet Cell Tumors – Functional tumors are hormone producing tumors. These tumors continue to produce the hormone the original cells were meant to create, like insulinomas and glaucagonomas. It is extremely rare for other hormone producing tumors to exist in the pancreas.
  • Gastrinoma – These tumors grow from pancreatic islet cells that create the hormone gastrin. This hormone triggers the release of stomach acid, which is used for digesting food.
  • Insulinoma – These tumors develop from the cells responsible for producing insulin. Insulin’s sole purpose is to control the amount of sugar in the bloodstream.
  • Glacagonoma – These tumors produce glucagonoma. This is the hormone responsible for triggering the liver to successfully release glucose into the bloodstream. 

Rare Functional Tumors

There are a few types of tumors that are considered functional islet cell tumors. However, these are extremely rare, even in comparison to the umbrella term that they are classified under. These rare tumors include:

Somatostatinomas – Releases the hormone somatostatin, which is responsible for determining and controlling the amount of sodium, water, and glucose that leaves the body.

What are the Symptoms of Islet Cell Cancer?

Islet cell tumors can cause a lot of different symptoms, all of which depend on the type of hormone producing cells that it develops from. Usually, the symptoms presented by nonfunctional tumors are created by the tumor metastasizing or growing.

For patients who are experiencing functional tumors, the symptoms produced will be determined by the type of hormone that it is secreting.

Symptoms of Nonfunctional Tumors

  • Increased frequency of gas
  • Increase frequency of indigestion
  • Frequent diarrhea
  • Pain in the abdomen
  • Pain in the back
  • An abdominal mass, possibly large enough to be felt from outside the abdomen.
  • Noticeable yellowing of the whites of the eyes and the skin.

Symptoms of Gastrinomas

  • A recurring stomach ulcer
  • Pain in the abdomen, may respond to antacids
  • Contents of the stomach coming back up into the esophagus
  • Frequent diarrhea

Symptoms of Insulinomas

  • Headaches
  • Lightheaded
  • Blurry Vision
  • Unexplained weakness
  • Unexplained shaking
  • Irritability
  • Feeling hungry

Islet Cell Cancer Causes

The exact cause of this rare hormone producing cancer is unknown, but the link to this disorder is hereditary. Family histories with multiple endocrine neoplasia syndromes prompt the endocrine glands to develop malignant and benign growths.

Genetics refer to MEN1, the genetic code for a gene mutation called menin, present in a family history of this disease. It triggers the release of hormones affecting specific body processes, increasing the risk of developing islet cell cancers, also referred to as pancreatic neuroendocrine tumors.

Two types of islet cell cancers are gastrinoma and glucagonoma, both of which are caused by an overproduction of hormones. Gastrinomas over secrete the hormone gastrin, leading to the production of gastric acids and stomach ulcers. Glucagonoma releases large qualities of the hormone glucagon, controlling blood sugar levels connected to diabetes.

How is Islet Cell Cancer Treated?

The most common treatment for malignant tumors is surgical removal. Many times, surgeons are unable to remove the entire tumor, but they do remove as much as possible so that chemotherapy and radiation are more effective.

Benign tumors are usually left alone, unless they cause severe symptoms or pain. Medications are usually prescribed to control the symptoms the tumor produces. Most functional tumors fall into the category of benign tumors.

Islet Cell Cancer Prevention

Premature islet cell cancers are curable if detected in early stages because endocrine neoplasms grow much slower than customary pancreatic cancers. If you have a family history of this disease or the underlying effects from other illnesses, you need to talk with your doctor at once.

As a mutated gene carrier, genetics put you at risk, but it do not predict the occurrence. You need to learn as much about this condition as possible, gather all the family information and allow your doctor to review the medical records.

The more you understand about the disorder, the better prepared you are to have a discussion with your doctor. Your family needs to be educated; family counseling and blood tests can help in determining the level of risk within the family bloodlines. The tests help to identify and measure the levels of hormones in the blood, the islet cell cancers predominate with your genetics and the best prevention strategies.

Last Reviewed:
October 06, 2016
Last Updated:
March 14, 2018