Appendix Cancer Overview

What is the appendix?

The appendix is a slender worm-like tube that is attached to the first section of the large intestine (known as the cecum).  The appendix is about three to six inches long. It is considered part of the gastrointestinal tract, though it is thought to have no digestive function. 

What is appendix cancer?

Cancer of the appendix is a rare kind of cancer. It occurs when cells in the appendix become abnormal and grow. These cells together form a growth of tissue, called a tumor. A tumor can be noncancerous (benign) or cancerous (malignant).  If cancerous, the tumor cells can spread to other parts of the body (metastasize). Another name for this type of cancer is appendiceal cancer.

Risk factors

A risk factor is anything that increases a person’s chance of developing a specific cancer. There are no known risk factors for cancer of the appendix. This type of cancer usually causes no symptoms until it has spread to other organs (metastasized). In fact, cancers of the appendix are often found incidentally when someone has their appendix taken out for symptoms of appendicitis or during another surgery in the abdomen such as a hernia repair. Appendix cancers are found in approximately 1% of all appendectomies.           

Types of appendix cancer

There are different kinds of tumors that can begin in the appendix. The most common type of tumor cells found on the appendix is carcinoid.  In a recent series, about 60 percent of all tumors in the appendix are carcinoid tumors1( Connor, SJ, Hanna, GB, Frizelle, FA, Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7970 appendectomies. Dis Colon Rectum 1998; 41:75.). Carcinoid tumors are a type of tumor that is also known as neuroendocrine tumor. They can arise in different locations throughout the body. Carcinoid tumors of the appendix commonly do not cause symptoms, and the treatment of a carcinoid tumor of the appendix that has not spread is to have it removed surgically. 

Other types of tumors found in the appendix include those that have spread from another site of cancer, or a “secondary tumor” (15%), appendix adenocarcinoma, which is treated similarly to colon cancers (11%), and mucinous cystadenomas, which are benign tumors of the appendix (9%).


Cancer of the appendix is usually asymptomatic and found for the first time when a patient has treatment for symptoms of appendicitis or during a surgical procedure such as hernia repair. It also can be found when a patient is treated for unexplained bloating around the abdomen. There are some other common signs and symptoms to watch for when it comes to cancer of the appendix. Common symptoms may include fatigue, loss of appetite and weight loss. Other symptoms may include:

  • quickly feeling full after eating
  • difficult bowel movements
  • frequent urination
  • shortness of breath
  • an increased heart rate
  • abdominal and lower back pain
  • indigestion, reflux, gas, nausea and/or vomiting

Treatment options

Treatment of cancer of appendix depends on the size of the tumor and whether cancer cells have spread to other parts of the body. If caught at an early stage, appendiceal cancer may be treated with surgery alone. Types of surgeries include appendectomy and hemicolectomy.

  • An appendectomy is the removal of the appendix and is usually the only treatment needed if the tumor is smaller than two centimeters. 
  • A hemicolectomy is the removal of a portion of the colon next to the appendix, nearby blood vessels and lymph nodes. This is usually used for a tumor larger than two centimeters.

Treatment for some cancers of the appendix may also include a combination of surgery along with chemotherapy.  During surgery, chemotherapy can be delivered into the open abdomen to help kill any tumor cells that have stayed behind after surgery. The chemotherapy is given by bathing all areas of the abdominal cavity. The surgery can involve removing involved organs such as part of the intestines, gallbladder, spleen, ovaries, uterus, or others. Patients may also want to consider participation on a clinical trial. A clinical trial is meant to obtain information on new treatments for patients with cancer. It is important to discuss your options with your medical oncologist and treatment care team and together decide which treatment is right for you.