Appendix cancer is a form of gastrointestinal carcinoid tumor, which is cancer that forms in the lining of the gastrointestinal tract.
The gastrointestinal tract includes the stomach, small intestine, and large intestine. These organs are part of the digestive system, which processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) in foods that are eaten and helps pass waste material out of the body.
Gastrointestinal carcinoid tumors develop from a certain type of hormone-making cell in the lining of the gastrointestinal tract. These cells produce hormones that help regulate digestive juices and the muscles used in moving food through the stomach and intestines. A gastrointestinal carcinoid tumor may also produce hormones. Carcinoid tumors that start in the rectum (the last several inches of the large intestine) usually do not produce hormones.
Gastrointestinal carcinoid tumors grow slowly. Most of them occur in the appendix (an organ attached to the large intestine), small intestine, and rectum. It is common for more than one tumor to develop in the small intestine. Having a carcinoid tumor increases a person's chance of getting other cancers in the digestive system, either at the same time or later.
The hormones produced by gastrointestinal carcinoid tumors are usually destroyed by blood and liver enzymes. If the tumor has spread to the liver, however, high amounts of these hormones may remain in the body and cause the following group of symptoms, called carcinoid syndrome:
· Redness or a feeling of warmth in the face and neck.
· Shortness of breath, fast heartbeat, tiredness, or swelling of the feet and ankles.
· Pain or a feeling of fullness in the abdomen.
These symptoms and others may be caused by gastrointestinal carcinoid tumors or by other conditions. Consult a doctor if any of these symptoms occur.
Different types of treatment are available for patients with gastrointestinal carcinoid tumors. Some treatments are standard (the currently used treatment, often referred to as “standard of care”), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Seven types of standard treatment are used:
Treatment of gastrointestinal carcinoid tumors usually includes surgery. One of the following surgical procedures may be used:
· Appendectomy: Removal of the appendix.
· Fulguration: Use of an electric current to burn away the tumor using a special tool.
· Cryosurgery: A treatment that uses an instrument to freeze and destroy abnormal tissue, such as carcinoma in situ. This type of treatment is also called cryotherapy. The doctor may use ultrasound to guide the instrument.
· Resection: Surgery to remove part or all of the organ that contains cancer. Resection of the tumor and a small amount of normal tissue around it is called a local excision.
· Bowel resection and anastomosis: Removal of the bowel tumor and a small section of healthy bowel on each side. The healthy parts of the bowel are then sewn together (anastomosis). Lymph nodes are removed and checked by a pathologist to see if they contain cancer.
· Radiofrequency ablation: The use of a special probe with tiny electrodes that release high-energy radio waves (similar to microwaves) that kill cancer cells. The probe may be inserted through the skin or through an incision (cut) in the abdomen.
· Hepatic resection: Surgery to remove part or all of the liver.
· Hepatic artery ligation or embolization: A procedure to ligate (tie off) or embolize (block) the hepatic artery, the main blood vessel that brings blood into the liver. Blocking the flow of blood to the liver helps kill cancer cells growing there.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy).
Chemoembolization of the hepatic artery is a type of regional chemotherapy that may be used to treat a gastrointestinal carcinoid tumor that has spread to the liver. The anticancer drug is injected into the hepatic artery through a catheter (thin tube). The drug is mixed with a substance that embolizes (blocks) the artery, cutting off blood flow to the tumor. Most of the anticancer drug is trapped near the tumor and only a small amount of the drug reaches other parts of the body. The blockage may be temporary or permanent, depending on the substance used to block the artery. The tumor is prevented from getting the oxygen and nutrients it needs to grow. The liver continues to receive blood from the hepatic portal vein, which carries blood from the stomach and intestine.
The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Percutaneous ethanol injection
Percutaneous ethanol injection is a cancer treatment in which a small needle is used to inject ethanol (alcohol) directly into a tumor to kill cancer cells. This procedure is also called intratumoral ethanol injection.
Biologic therapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.
Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances produced by glands in the body and circulated in the bloodstream. The presence of some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy are used to reduce the production of hormones or block them from working.
Other drug therapy
MIBG (metaiodobenzylguanidine) is sometimes used, with or without radioactive iodine (I131), to lessen the symptoms of gastrointestinal carcinoid tumors.