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The information on this page was reviewed and approved by
Maurie Markman, MD, President, Medicine & Science at CTCA.

This page was updated on April 2, 2021.

About appendix cancer

Appendix cancer is rare. The American Cancer Society does not break out separate incidence rates for the disease in the United States. Instead, appendix cancer is part of the cancer statistics for digestive organs. Sometimes called gastrointestinal carcinoid tumors, these cancers may develop in the stomach, small intestine, colon, rectum or appendix.

The appendix is attached to the first section of the large intestine. Appendix tumors form when healthy cells in the appendix mutate and multiply uncontrollably. Malignant (cancerous) tumors may spread cancer cells to other parts of the body.  Patients may have better outcomes if the cancer has not spread beyond the appendix.

At Cancer Treatment Centers of America® (CTCA), our oncologists use an integrative approach to attack appendix cancer. We rely on evidence-based medical treatments, while also helping patients manage their physical and psychological side effects. For appendix cancer patients, the side effects of treatment may include loss of appetite, fatigue and nausea.

What causes appendix cancer?

Doctors do not know what causes appendix cancer, the American Society for Clinical Oncology (ASCO) says. No avoidable risk factors have been identified.

Learn more about risk factors for appendix cancer

Who gets appendix cancer?

An individual’s family history may play a role in appendix cancer. Individuals are at higher risk if a relative has been diagnosed with:

  • Appendix cancer
  • Multiple endocrine neoplasia type 1 (MEN1) syndrome, also known as endocrine adenomatosis or Wermer syndrome

Medical conditions that affect acid production in the stomach may also increase risk of the disease. Those conditions may include atrophic gastritis, pernicious anemia and Zollinger-Ellison syndrome.

Women are more likely than men to develop carcinoid tumors, one type of appendix cancer. Smokers are also more likely to develop appendix cancer than non-smokers.

The average age at diagnosis is between 50 and 55 years old, affecting men and women equally. The disease is rare in children.

Appendix cancer types

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 symptoms

Cancers of the appendix typically do not cause symptoms until they have reached an advanced stage. Symptoms may include:

  • Appendicitis
  • Ovarian masses
  • Loss of appetite
  • Bloating or increase in abdominal girth
  • Changes in bowel function
  • Abdominal pain, particularly in the lower right side of the abdomen
  • Indigestion
  • New hernias
  • Reflux
  • Vomiting

Learn more about symptoms of appendix cancer

Diagnosing appendix cancer

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 CTCA® use for appendiceal cancer include:

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

Learn more about diagnostic procedures for appendix cancer

Treating appendix cancer

Appendix cancer treatment options typically involve surgery or surgery in combination with chemotherapy.

Types of chemotherapy used to treat tumors of the appendix may include:

  • Systemic chemotherapy
  • Hyperthermic intraperitoneal chemotherapy (HIPEC)
  • Intraperitoneal chemotherapy (EPIC)
  • A combination of both systemic and regional chemotherapies

Surgical treatments depend on the types of tumors and their locations. Procedures may include:

  • A hemicolectomy, which removes a portion of the colon near the appendix along with nearby lymph nodes and blood vessels
  • Cytoreductive surgery, also known as debulking surgery, which involves surgical removal of as much of the tumor bulk as possible
  • A peritonectomy, which removes the peritoneum (the lining of the abdominal cavity)

Learn more about treatment options for appendix cancer