Appendix Cancer

A very rare type of cancer affecting the appendix, a pouch-like tube of about 4 inches, is called appendix cancer. The appendix is a part of the gastrointestinal tract and has no significant function. When the healthy cells in the appendix grow uncontrollably, they constitute a tumor that may be cancerous or benign. Appendiceal cancer is mainly classified into different types as follows:

• Epithelial appendiceal cancer – It grows from the cells that make up the lining of the appendix and leads to a build-up of mucin that eventually causes the appendix to rupture.
• Neuroendocrine appendiceal cancer or carcinoid tumors – In this type, the tumor cells grow from the enterochromaffin cells. It accounts for over half of appendix cancer but can be treated with surgery.
• · Appendix adenocarcinoma – It begins from the cells that line the inside of the appendix and behaves like the most common type of colorectal cancer.
• Goblet cell carcinomas/adenocarcinoids – These tumors have the features of both adenocarcinomas and neuroendocrine tumors but are more aggressive than the latter. The treatment for this type of cancer is similar to that used for adenocarcinoma.

Causes

While the exact cause is unknown, several factors increase the risk of developing it. These include:

• · Family history – Patients whose relatives were diagnosed with appendix cancer or multiple endocrine neoplasia type 1 (MEN1) syndrome are at a higher risk.
• · Other diseases – Atrophic gastritis, pernicious anemia, and Zollinger-Ellison syndrome increase the risk.
• · Gender – Women are more likely to develop appendix cancer than men.
• · Age – The risk increases with age, and most people are often diagnosed in their 40s.
• · Smoking or other tobacco products – Smokers are more likely to get appendix cancer than non-smokers.

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Symptoms of Appendix Cancer

Appendix cancer is mostly asymptomatic until it spreads to other organs making it difficult to detect in the early stages. Some of the warning signs in patients are:

• · Appendicitis,
• · Bloated abdomen,
• · Chronic or severe abdominal pain,
• · Pelvic pain,
• · Ascites (fluid build-up in the abdomen),
• · Changes in bowel function,
• · Nausea, vomiting, indigestion,
• · Increase in waist size,
• · Fertility problems,
• · Ovarian masses,
• · Non-specific discomfort in the lower right abdomen,
• · New hernias.

Screening tests

The diagnosis is often made by chance. It is generally identified during the testing or treatment of other health conditions or surgery for Appendicitis. Some of the common diagnostic procedures that help in screening appendiceal cancer are:

Ø Imaging: Radiological studies like CT and MRI scans can be used to see how deep cancer has grown into the tissues of the appendix and check for metastasis.

Ø Diagnostic Laparoscopy: In this procedure, a laparoscope is inserted into the abdomen via an incision to visualize the organs in the area. If any signs of tumors are observed, then a biopsy is recommended for the patient.

Ø Biopsy: In a biopsy, a small piece of the tumor cells or tissues is removed by a fine needle and observed under a microscope by a

pathologist. It is usually challenging to perform biopsies of the appendix, so they are often done on the tissues in the body where cancer has metastasized.

Treatment of Appendix Cancer

The treatment protocol is uniquely tailored to each patient depending on the type of tumor and the overall health condition. The various types of treatment options available for appendix cancer are:

· Surgery – Surgery is the most common treatment for localized appendiceal cancer. If the cancer is localized to the appendix only, the surgical intervention involves removing the entire appendix (appendectomy). In some cases, when the cancer is more significant, surgery is done to remove one-half of the colon (hemicolectomy). However, when cancer has metastasized, a cytoreductive surgery (debulking surgery), which involves the removal of the tumor, surrounding fluid, and any nearby organ attached to the tumor, is recommended.

· Chemotherapy – Drugs are administered to patients before or after surgery. Different types of chemotherapy used for the palliative care of appendiceal cancer patients are:

• § Systemic chemotherapy
• § Regional chemotherapy like hyperthermic intraperitoneal chemotherapy
• § Combination of systemic and regional chemotherapy.

· Radiation therapy – High-powered beams such as x-rays or protons are used to kill the cancer cells or prevent their metastasis.