Pancreatic Neuroendocrine Tumors – Symptoms and Causes

Overview

Pancreatic neuroendocrine tumors are uncommon cancers that form in the pancreas. The pancreas is a flat organ behind the stomach that makes digestive enzymes and hormones.

These tumors grow from the hormone-making islet cells in the pancreas. Doctors sometimes call them islet cell cancers.

Pancreatic neuroendocrine tumors come in two main types:

  • Functional Tumors: These keep making hormones and produce too much of them. Examples include:
    • Insulinoma
    • Gastrinoma
    • Glucagonoma
  • Nonfunctional Tumors: Most pancreatic neuroendocrine tumors fall into this group. They don’t make extra hormones.

The symptoms a person has depend on whether their tumor is functional or nonfunctional, and which hormones might be affected. Each type causes different health problems based on the specific hormone involved.

Signs and Symptoms

People with pancreatic neuroendocrine tumors may not always have symptoms. When symptoms do appear, they can include:

  • Heartburn and indigestion
  • Feeling weak or tired
  • Muscle cramps
  • Diarrhea or constipation
  • Unexplained weight loss
  • Skin rashes
  • Pain in the stomach or back
  • Yellow skin and eye whites
  • Dizziness and headaches
  • Blurry vision
  • Increased thirst and hunger

When You Should Contact a Doctor

If you notice any of these symptoms and they worry you, it’s important to schedule an appointment with your healthcare provider. Early detection can help with treatment options.

Causes

Pancreatic neuroendocrine tumors begin when DNA changes occur in the hormone-producing islet cells of the pancreas. These DNA mutations give faulty instructions to cells, causing them to:

  • Multiply rapidly without control
  • Continue living when they should naturally die
  • Form masses called tumors
  • Sometimes spread to other organs like the liver (metastasis)

The exact triggers for these DNA changes remain unknown. When cells experience these mutations, they no longer follow normal growth patterns. Instead, they accumulate and can develop into tumors.

In healthy cells, DNA provides the blueprint for normal cell function and lifecycle. However, when mutations happen in the pancreatic islet cells, this process becomes disrupted.

The abnormal cells may continue to produce hormones, but they do so in an unregulated manner.

Risk Factors

Several factors may increase your chance of developing pancreatic neuroendocrine tumors:

Family History
If someone in your family has had this type of tumor, you have a higher risk of developing one yourself. This family connection is important to consider when assessing your personal risk profile.

Inherited Syndromes
Certain genetic conditions that pass from parents to children can raise the risk of these tumors. These inherited syndromes happen because of changes in DNA that allow cells to grow and divide more than they should. Examples include:

  • Multiple Endocrine Neoplasia, type 1 (MEN 1)
  • Von Hippel-Lindau (VHL) disease
  • Neurofibromatosis 1 (NF1)
  • Tuberous Sclerosis

These conditions are present from birth and can affect various parts of the body, not just the pancreas.

It’s important to understand that if you develop a pancreatic neuroendocrine tumor, you did not cause it. There are currently no known prevention methods for this type of cancer.

When doctors evaluate patients with pancreatic symptoms, they often look at family medical history to determine if genetic testing might be helpful. People with the inherited syndromes listed above typically receive more frequent monitoring to catch any tumors early.

Research continues to explore other possible risk factors, but currently, these genetic and family-related factors are the main known risks for pancreatic neuroendocrine tumors.


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