August is Appendix Cancer Awareness Month. Appendix cancer also called appendiceal cancer, is very rare, affecting about 1 or 2 people per 1 million per year. It is more common in people over the age of 50.
Appendix cancer develops when healthy cells in your appendix mutate and grow out of control, forming a tumor. It’s usually found during surgery for appendicitis or when an imaging test is taken for an unrelated condition. Smaller tumors are less likely to spread, while larger tumors generally require aggressive treatment.
There are two main types of appendiceal cancer: appendiceal epithelial cancer and neuroendocrine appendiceal cancer.
Epithelial appendiceal cancer grows from cells that make up the lining of the appendix. It may also be called adenocarcinoma. These cells help make a jelly-like substance called mucin. Mucin protects the lining of the stomach, intestines, and appendix. Most epithelial appendiceal cancers can lead to a build-up of mucin, which can cause the appendix to rupture. When mucin from the appendix builds up in the abdomen, it causes pseudomyxoma peritonei or PMP.
Mucinous neoplasms are a rare and complex type of appendiceal epithelial cancer. Depending on how the cells look under a microscope, they may be low-grade (LAMN) or high quality (HAMN) and may spread to other body parts.
Neuroendocrine tumors of the appendix grow from enterochromaffin cells (ECs). ECs make chemicals involved in digestion and movement in the intestines. Neuroendocrine tumors of the appendix are the most common type of appendiceal cancer. They are also called carcinoid tumors.
Appendiceal cancer can spread to different parts of the abdomen directly from the appendix, usually when it ruptures. Less often, appendiceal cancer can spread through lymph nodes to areas outside the abdomen.
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Symptoms can include:
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Pain
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Feeling bloated or growing abdominal size
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A mass in the abdomen
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Nausea and vomiting
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Feeling full soon after starting to eat
How is appendiceal cancer treated?
Treatment for each patient is individualized and may include surgery or chemotherapy.
Surgery: You may have surgery to remove the appendix and any other organs and tissue in the abdomen and pelvic area that may be affected. Affected areas may include parts of your intestine, colon, and peritoneum.
Chemotherapy: When appendiceal cancer has spread to other parts of the abdomen, chemotherapy is used to kill the cancer cells. In some cases, a specific way of giving chemotherapy, called hyperthermic intraperitoneal chemotherapy, or HIPEC, is used. This treatment uses hot chemicals to wash the inside of the abdomen during surgery.
Appendiceal Cancer Screening
No standardized process or testing exists to diagnose or screen for appendix cancer. If your doctor suspects you may have appendix cancer, they will order further tests to help make an accurate diagnosis, such as imaging tests or a biopsy. Imaging tests can reveal the presence of tumors on your appendix, while a biopsy removes small tissue samples to test for cancerous cells.