Appendix cancer

What is appendix cancer?

Cancer of the appendix is a rare form of cancer which grows in the appendix. There are many different forms of appendix cancer, and in the early stages, a person frequently experiences no symptoms.

The appendix is a tubular, about 4 inches in length, finger-like sac that attaches to the first part of the colon. The precise function of this organ is not well known by scientists. Without an appendix, people will live normal and healthy lives.

Cancer of the appendix, sometimes called cancer of the appendix, is very rare. Experts estimate that about 2 to 9 individuals per 1 million are affected by this form of cancer. Some reports indicate that however, appendix cancer may be on the increase.

A recent retrospective study reported that it increased from about 6 per 1 million individuals in 2000 to as much as 10 per 1 million individuals in 2009.

We address the forms, symptoms, causes, and risk factors of appendix cancer in this article. Diagnosis, recovery, and survival rates for this condition are also covered.


Types of appendix cancer

In the appendix, there are different types of tumors that can start:

  • Neuroendocrine tumor : In hormone-producing cells, a neuroendocrine tumor begins and is usually found in small quantities in almost every organ in the body. This can be called a carcinoid tumor as well. In general, a neuroendocrine tumor begins in either the GI tract or lungs, but can also occur in the pancreas, testicles, or ovaries. A neuroendocrine tumor in the appendix most commonly occurs at the tip of the appendix. Neuroendocrine tumors account for about 50 percent of all appendix tumors. Appendix cancer typically does not cause any symptoms before it has spread to other organs and sometimes goes unnoticed before it is detected for some purpose during an examination or operation. A neuroendocrine tumor in the appendix, which remains limited to the area where it began, has a high probability of successful surgical treatment. Read more about GI tract neuroendocrine tumors.
  • Appendiceal mucoceles : Swelling or sacs from swelling of the appendix wall, usually filled with mucous membranes, are mucoceles. There are a number of benign to malignant conditions that may arise to form a mucocele in the appendix. Mucinous cystadenomas and mucinous cystadenocarcinomas constitute two of these disorders. Mucinous cystadenomas are benign and do not spread, and adenomatous polyps that may form in the colon are similar. They can be removed entirely with surgery as they are found in the appendix. However if the appendix splits, the cells will spread through the cavity of the body and proceed to secrete mucin, a jelly-like material, into the abdomen. Mucin accumulation can lead to abdominal pain, bloating and bowel function changes, including bowel obstruction (blockage). Mucinous cystadenocarcinomas with mucin in the abdomen can have similar symptoms, but they are malignant, which means they can spread to other areas of the body.
  • Colonic-type adenocarcinoma : Adenocarcinoma of the colonic form accounts for about 10 percent of appendix tumors and typically occurs at the appendix base. The most common form of colorectal cancer looks and acts like appendix cancer. It often goes unnoticed, and for appendicitis, a diagnosis is often made during or after surgery. Appendicitis is appendix inflammation that may cause abdominal pain or swelling, loss of appetite, nausea, vomiting, constipation or diarrhea, gas inability to pass through or low fever that starts after other symptoms have formed.
  • Signet-ring cell adenocarcinoma : Signet-ring cell adenocarcinoma is rare and is known to be more severe than other forms of adenocarcinoma and more difficult to treat. It is called adenocarcinoma of signet-ring cells because the cell appears like it has a signet ring inside it under the microscope. Similar to colon cancer, this form of appendix cancer is also treated as well.
  • Goblet cell carcinomas/Adenoneuroendocrines : Both adenocarcinomas and neuroendocrine tumors have characteristics of goblet cell carcinomas. They are more aggressive than neuroendocrine tumors and are mostly treated in a similar way to adenocarcinoma treatment.
  • Paraganglioma : This is a rare tumor that grows from paraganglia cells, a collection of nerve tissue-derived cells that survive during fetal (pre-birth) growth in small deposits. Paraganglia is also present near the adrenal glands and in the head and neck regions of the body, including several blood vessels and nerves. This type of tumor is commonly considered benign and is mostly treated successfully with the complete removal of the tumor by surgery. Learn about paraganglioma more.

 Symptoms of appendix cancer

At the beginning, appendix cancer does not have any visible signs. Typically it is observed for another disorder like appendicitis during surgery or during an imaging examination.

During a regular colonoscopy, the doctor may even notice it. If there are signs, however they can include:

  • bloated abdomen
  • ovarian masses
  • chronic or severe abdominal pain
  • nonspecific discomfort in the lower right abdomen
  • obstruction of the bowel
  • hernia
  • diarrhea

Risk factors for appendix cancer

Although some experts note that no risk factors for the development of appendix cancer have been confirmed, a few possible ones have been suggested.

These include:

  • pernicious anemia, a deficiency of vitamin B-12
  • atrophic gastritis, or long-term inflammation of the stomach lining
  • Zollinger-Ellison syndrome, a condition of the digestive tract
  • a family history of multiple endocrine neoplasia type 1 (MEN1), a disorder that leads to tumors in the glands that produce hormones
  • smoking

Treatment of appendix cancer

The treatment for appendix cancer depends on the:

  • type of tumor
  • stage of the cancer
  • person’s overall health

The most popular treatment for localized appendix cancer is surgery. If the cancer is present only in the appendix, then the treatment usually includes removing the appendix. This is called an appendectomy as well.

Your doctor may suggest removing one half of your colon and even some lymph nodes for some forms of appendix cancer, or if the tumor is larger. A hemicolectomy is called surgery to remove half of the colon.

If the cancer has spread, then cytoreductive surgery, often called debulking, could be advised by your doctor. The surgeon would remove the tumor, surrounding fluid, and probably any neighboring organs that are connected to the tumor in this form of surgery.

Treatment may include chemotherapy before or after surgery if:

  • the tumor is larger than 2 centimeters
  • the cancer has spread, especially to the lymph nodes
  • the cancer is more aggressive

Types of chemotherapy include:

  • systemic chemotherapy, given intravenously or by mouth
  • regional chemotherapy, given directly into the abdomen, such as intraperitoneal chemotherapy (EPIC) or hyperthermic intraperitoneal chemotherapy (HIPEC)
  • a combination of systemic and regional chemotherapies

Afterward, your doctor will follow up with imaging tests, such as a CT scan or MRI, to ensure the tumor is gone.

What’s the recurrence and survival rate?

According to a 2011 review trusted source, 5-year survival rates for appendix cancer after the appendix was removed are:

  • 94 percent if the carcinoid tumor is confined to the appendix
  • 85 percent if the cancer has spread to lymph nodes or nearby areas
  • 34 percent if the cancer has spread to distant organs, but this is very rare for carcinoid tumors

The 5-year survival rate increases for some cases of appendix cancer when part of the colon is also removed and chemotherapy is used. However, not all cases of appendix cancer require these additional treatments.

Chemotherapy in appendix cancer

Based on the delivery process, the major forms of chemotherapy for appendix cancer are classified. They include:

  • Local (intraperitoneal) chemotherapy : The surgeon inserts a tube into the abdominal cavity of the patient to allow chemotherapy to be administered directly to the target area after a surgeon performs debulking surgery to extract as much of a tumor as possible.
  • Hyperthermic (heated) intraperitoneal chemotherapy (HIPEC) :– The drugs are warmed to a temperature that reaches the body temperature of the patient prior to delivery; this will improve the chemo’s ability to penetrate tissue that contains tumor cells.
  • Systemic chemotherapy : The treatments are delivered by mouth or intravenously so that the chemo can penetrate the bloodstream of a patient to reach cancerous cells that have spread across his or her body.

The combinations of chemotherapy drugs most commonly used when systemic chemotherapy is indicated for appendiceal cancer are:

    • FOL– Folinic acid (Leucovorin)
    • F– Fluorouracil (5FU)
    • OX – Oxaliplatin (Eloxatin)
    • FOL– Folinic Acid (Leukovorin)
    • F– Fluorouracil (5FU)
    • IRI- Irinotecan (Camptosar)
  3. XELOX
    • XEL– Xeloda (Capecitabine)
    • OX– Oxaliplatin

Other drugs that may be added to these regimens:

  • Avastin (Bevacizumab): a monoclonal antibody that interferes with a tumor’s ability to form blood vessels
  • Erbitux (Cetuximab): a monoclonal antibody used colorectal cancer
  • Xaliproden (SR57746A)- a new drug currently being tested in clinical trials for the prevention of neuropathy in patients receiving oxaliplatin.

Radiation therapy in appendix cancer

Using high-energy x-rays or other particles to kill cancer cells is radiation therapy. A doctor who specializes in treating cancer by giving radiation therapy is called a radiation oncologist. A regimen (schedule) of radiation therapy typically consists of a fixed number of treatments provided over a specified period of time.

In the treatment of appendix cancer, radiation therapy using a schedule is seldom used. However to help alleviate symptoms, it may also be used to treat a specific region when the cancer has spread, such as the bone (see below.)

Fatigue, mild skin reactions, upset stomach, and loose bowel movements can include side effects from radiation therapy. Soon after treatment is over, most side effects go away.

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  • September 2nd, 2020

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