Appendix cancer causes and risk factors

This page was reviewed under our medical and editorial policy by

Maurie Markman, MD, President, Medicine & Science

This page was updated on June 8, 2022.

Appendix cancer is an extremely rare disease, diagnosed in just one or two people per million in the United States annually. Researchers believe it's becoming more common and are studying why.

What causes appendix cancer?

The exact cause of appendix cancer isn't known. This disease develops when cells within the appendix change and grow out of control, resulting in the formation of a tumor.

Risk factors for appendix cancer are varied, but often involve lifestyle habits, age, genes and medical history. Having a risk factor for cancer does not mean a person will get the disease. Some people who have multiple risk factors never develop appendix cancer, while others get the disease despite no known risk factors.

Is appendix cancer hereditary?

Patients with a relative who has or has had appendix cancer or multiple endocrine neoplasia type 1 (MEN1) syndrome (also known as endocrine adenomatosis or Wermer syndrome) have a higher risk for developing this disease.

Appendix cancer risk factors

Risk factors for developing appendiceal cancer include those listed below.

Smoking: Smokers are more likely to develop appendix cancer than non-smokers.

Medical history: Those with a history of certain medical conditions that affect the stomach’s ability to produce acid, such as atrophic gastritis, pernicious anemia and Zollinger-Ellison syndrome, are at an increased risk for appendix cancer.

Gender: Women are more likely to develop carcinoid tumors than men.

Age: Most people diagnosed with appendix cancer are between 50 and 55 years old.

Next topic: What are the symptoms of appendix cancer?

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