The appendix is a tube that looks like a small sack or pouch. It’s connected to the colon near the beginning of the large intestine.The appendix doesn’t have a known purpose. However, it may have something to do with the immune system.Appendix cancer is sometimes called appendiceal cancer. It occurs when healthy cells become abnormal and grow rapidly. These cancerous cells become a mass or tumor inside the appendix. When the tumor is malignant, it’s considered cancerous.

Appendix cancer is considered very rare.There are different classifications of appendix cancers that aren’t well-defined. The lack of well-defined classifications is due to the rarity of this type of cancer, which limits the amount of research.

Types of appendix tumors

There are different types of tumors that can start in the appendix:

• Neuroendocrine tumor. A neuroendocrine tumor starts in the hormone-producing cells that are normally present in small amounts in almost every organ in the body. This may also be called a carcinoid tumor. A neuroendocrine tumor usually starts in either the GI tract or lungs, but it also may occur in the pancreas, testicles, or ovaries. An appendix neuroendocrine tumor most often occurs at the tip of the appendix. Approximately 50% of all appendix tumors are neuroendocrine tumors

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• Appendiceal mucoceles. Mucoceles are swellings or sacs from swelling of the appendix wall, typically filled with mucous. There is a range of benign to malignant conditions that can occur in the appendix to form a mucocele. Two of these conditions are mucinous cystadenomas and mucinous cystadenocarcinomas. Mucinous cystadenomas are benign and do not spread and they are similar to adenomatous polyps that can develop in the colon

• Colonic-type adenocarcinoma. Colonic-type adenocarcinoma accounts for about 10% of appendix tumors and usually occurs at the base of the appendix. Appendix cancer looks and behaves like the most common type of colorectal cancer. It often goes unnoticed, and a diagnosis is frequently made during or after surgery for appendicitis.

• Signet-ring cell adenocarcinoma. Signet-ring cell adenocarcinoma is rare and considered to be more aggressive and more difficult to treat than other types of adenocarcinomas. It is called signet-ring cell adenocarcinoma because, under the microscope, the cell looks like it has a signet ring inside it.  This type of appendix cancer is also treated similar to colon cancer

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• Goblet cell carcinomas/Adenoneuroendocrines. Goblet cell carcinomas have features of both adenocarcinomas and neuroendocrine tumors. They are more aggressive than neuroendocrine tumors, and treatment is often similar to treatment for adenocarcinoma

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• Paraganglioma. This is a rare tumor that develops from cells of the paraganglia, a collection of cells that come from nerve tissue that persist in small deposits after fetal (pre-birth) development. Paraganglia is often found near the adrenal glands and some blood vessels and nervesincluding in the head and neck region of the body.

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