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What is Appendix Cancer

What is Appendix Cancer

Executive Summary

Appendix cancer is the cancer of the appendix that occurs due to abnormal growth of the healthy cells forming tumors inside the appendix, a component of the gastrointestinal system. An appendix is an almost 10 cm long pouch-like tube connecting the cecum, the first segment of the large intestine, to the colon. It is a possible site for different types of tumors. Tumors of the neuroendocrine system, mucoceles in the appendix, adenocarcinoma of the colon, adenocarcinoma of the signet ring cell type, goblet cell carcinomas, and paraganglioma are the most common type of tumors in case of appendix cancer.

What is Appendix Cancer?

The appendix is a pouch-like tube that connects the cecum, the first segment of the large intestine, to the colon. The appendix is about 10 cm long on average. It is also a component of the gastrointestinal (GI) system. Despite being counterintuitive, the appendix, which seemingly has no role in the body, could be a member of the lymphatic, exocrine, or endocrine systems. When healthy cells in the appendix alter and expand out of control, resulting in appendix cancer ?1?.

A tumor is a tissue growth that, in particular consists of these cells. Tumors can be malignant or non-cancerous. A malignant tumor has the potential to grow and eventually spread to other regions of the body. Appendiceal carcinoma is another name for appendix cancer. Benign tumor refers to a tumor that can develop but not spread.

Tumors of several sorts can begin in the appendix

Tumor of the neuroendocrine system:

A neuroendocrine tumor begins in hormone-producing cells, found in modest quantities in practically every body organ. A carcinoid tumor is another name for this type of tumor. Neuroendocrine tumors usually begin in the gastrointestinal system or the lungs, although they can also develop in the pancreas, testicles, or ovaries. The common location of an appendix neuroendocrine tumor is the tip of the appendix. As a matter of fact, neuroendocrine tumors make for around half of all appendix tumors ?2?. Appendix cancer usually does not cause symptoms until it has spread to other organs, and it often remains undiagnosed until it is discovered during a routine checkup or operation. A neuroendocrine tumor in the appendix that remains restricted to the location it began undoubtedly has a good chance of being treated successfully with surgery ?3?. Learn more about gastrointestinal neuroendocrine tumors.

Mucoceles in the appendix:

Mucoceles are mucous-filled swellings or sacs caused by a bulge of the appendix wall. Various benign to malignant diseases in the appendix can cause mucocele. Mucinous cystadenomas and mucinous cystadenocarcinomas are in fact two of these disorders. Mucinous cystadenomas are comparable to adenomatous polyps in the colon in that they are more than often benign and do not spread. Hence, they are eliminated with surgery if they are contained in the appendix. On the other hand, if the appendix ruptures, the cells may spread throughout the body and release a jelly-like substance called mucin into the belly. Mucin buildup can cause abdominal pain, bloating, and bowel function abnormalities, including bowel obstruction (blockage). Mucinous cystadenocarcinoma in the abdomen can have similar consequences to mucin, but they are malignant, which means they can spread to other regions of the body ?4?.

Adenocarcinoma of the colon:

Colonic-type adenocarcinoma accounts for roughly 10% of appendix cancers and is most commonly found at the appendixs base ?5?. However, Appendix cancer resembles the most common type of colorectal cancer in appearance and behavior. It generally goes unrecognized until the diagnosis after appendicitis surgery. Appendicitis is an inflammation of the appendix that causes abdominal discomfort, swelling, nausea, vomiting, constipation or diarrhea, inability to pass gas, and a low fever that appears in addition to other symptoms.

Adenocarcinoma of the signet ring cell type:

Signet-ring cell adenocarcinoma is an uncommon kind of adenocarcinoma that is more aggressive and difficult to cure than other adenocarcinomas ?6?. Its named signet-ring cell adenocarcinoma because the cell seems to have a signet ring inside it under a microscope. This sort of appendix cancer is also treated the same way ascolon cancer.

Adenoneuroendocrine carcinomas/Goblet cell carcinomas:

Adenocarcinomas and neuroendocrine tumors both have features of goblet cell carcinomas (both described above). They are more aggressive than neuroendocrine tumors, and treatment is frequently comparable to adenocarcinoma treatment ?7?.


A rare tumor arises from paraganglia cells, a collection of nerve tissue cells that persist in small deposits after fetal (pre-birth) development. Paraganglia can be found in the head and neck region of the body, near the adrenal glands, and also in some blood arteries and nerves. This form of tumor is usually benign, and it is of course treated effectively with total surgical removal. In other words, paraganglioma is a type of cancer that affects the nervous system.


  1. 1.
    Osueni A, Chowdhury Y. statpearls. Published online December 14, 2021.
  2. 2.
    Ruoff C, Hanna L, Zhi W, Shahzad G, Gotlieb V, Saif M. Cancers of the appendix: review of the literatures. ISRN Oncol. 2011;2011:728579. doi:10.5402/2011/728579
  3. 3.
    Lockhart M, Smith J, Canon C, Morgan D, Heslin M. Appendiceal ganglioneuromas and pheochromocytoma in neurofibromatosis type 1. AJR Am J Roentgenol. 2000;175(1):132-134. doi:10.2214/ajr.175.1.1750132
  4. 4.
    Van de, De H, Sagaert X, Van C. Appendiceal cancer?: a review of the literature. Acta Gastroenterol Belg. 2020;83(3):441-448.
  5. 5.
    Nitecki S, Wolff B, Schlinkert R, Sarr M. The natural history of surgically treated primary adenocarcinoma of the appendix. Ann Surg. 1994;219(1):51-57. doi:10.1097/00000658-199401000-00009
  6. 6.
    McGory M, Maggard M, Kang H, OConnell J, Ko C. Malignancies of the appendix: beyond case series reports. Dis Colon Rectum. 2005;48(12):2264-2271. doi:10.1007/s10350-005-0196-4
  7. 7.
    Pahlavan P, Kanthan R. Goblet cell carcinoid of the appendix. World J Surg Oncol. 2005;3:36. doi:10.1186/1477-7819-3-36
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