Appendix cancer

Although cancer research has not determined the causes of appendix cancer, certain factors may increase a person’s risk of developing the disease.

Risk factors include:

• Smoking
• Family history: 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.
• 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.
• Gender: Women are more likely to develop appendix cancer than men.
• Age: The average age at diagnosis is 40.

Learn more about risk factors for appendix cancer

There are two main types of appendix cancer: neuroendocrine (carcinoid) tumors and carcinomas.

Neuroendocrine tumors (NET) are the most common form of appendix cancer. This type of tumor begins in hormone-producing cells and is typically found after the removal of the appendix in a surgical procedure called an appendectomy.

Carcinomas begin in the tissue that lines the appendix. Carcinomas of the appendix include:

• Mucinous adenocarcinoma, which produces mucin, a jelly-like substance that spreads cancerous cells to other parts of the body
• Goblet cell carcinoma, also called goblet cell carcinoids or adenocarcinoid tumors (though they are not carcinoid tumors), which behave similarly to mucinous adenocarcinoma
• Intestinal-type adenocarcinoma, also called colonic-type adenocarcinoma, which is usually found near the base of the appendix
• Signet-ring cell adenocarcinoma, which often causes appendicitis when it develops in the appendix

In addition to tumors, mucinous neoplasms may form on the appendix. These growths are usually benign and include mucoceles, mucous-filled sacs that form along the appendix wall, and pre-cancerous lesions. When low-grade mucinous neoplasms rupture, which is rare, they can cause a condition called pseudomyxoma peritonei (PMP).

Learn more about appendix cancer types

Appendix cancer is often diagnosed by chance. It may show up during testing or treatment for other conditions, or during surgery for appendicitis.

The diagnostic tools doctors at City of Hope use for appendiceal cancer include:

• Laboratory tests, such as such as blood and urine samples, and advanced genomic testing to examine the DNA of tumor cells
• Ultrasound
• Magnetic resonance imaging (MRI)
• Computed tomography (CT) scan
• Positron emission tomography scan (PET scan)

Learn more about diagnostic procedures for gallbladder cancer

We recognize that treating your appendix cancer requires a multidisciplinary approach to care. That’s why your care team includes doctors and other clinicians with different types of expertise—all working together under one roof to develop and execute a treatment plan tailored to your needs. Your care team uses conventional treatments to attack the disease itself, as well as evidence-informed therapies to help combat cancer- and treatment-related side effects.

Because side effects of appendix cancer often include loss of appetite, fatigue and nausea, you may meet with a registered dietitian to help you evaluate your nutritional status and make recommendations for staying nourished with healthy foods and a well-balanced diet.

Dietitians at City of Hope are part of a larger nutrition therapy team focused on keeping your immune system strong, preventing malnutrition and supporting your recovery from cancer treatments.