National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Adenocarcinoma of the appendix


Other Names:
Mucinous adenocarcinoma; Cystadenocarcinoma; Nonmucinous adenocarcinoma; Mucinous adenocarcinoma; Cystadenocarcinoma; Nonmucinous adenocarcinoma; Colonic type adenocarcinoma See More
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Cancer of the appendix is very rare and is typically found incidentally during appendectomies, in about 1% of the cases. According to a report published by the National Cancer Institute, using the Surveillance, Epidemiology, and End Results (SEER) database, appendix cancer account for about 0.4% of gastrointestinal tumors. There are several subytpes. The most common is the carcinoid type (66% of the total), with cyst-adenocarcinoma accounting for 20% and adenocarcinoma accounting for 10%. Then there are the rare forms of cancers which include adenocarcinoid, signet ring, non-Hodgkin’s lymphoma, ganglioneuroma, and pheochromocytoma. Benign primary tumors are mainly “mucinous epithelial neoplasms”, also called adenomas, cystadenoma, and benign neoplastic mucocele.[1]

Adenocarcinoma of the appendix is a epithelial cancer of the appendix.[2] The term 'epithelium' refers to cells that line hollow organs and glands and those that make up the outer surface of the body. Epithelial cells help to protect or enclose organs. Some produce mucus or other secretions.[3] Types of adenocarcinoma of the appendix include mucinous adenocarcinoma, non-mucinous adenocarcinoma, and signet cell carcinoma of the appendix (which is the rarest, involving only 4% of all the subtypes of appendix cancer).[2][4]
Last updated: 6/12/2015
The most common symptom of adenocarcinoma of the appendix is acute appendicitis.[2][5][6] Other symptoms include a palpable abdominal mass, ascites (fluid buildup), peritonitis (inflammation of the membrane lining the abdominal cavity) due to a hole or tear in the appendix, and non-specific gastrointestinal or genitourinary symptoms such as bloating, vague abdominal pain, and tenderness.[4][5]
Last updated: 12/7/2016
The diagnosis of adenocarcinoma of the appendix is rarely made or suspected without surgery. It is often identified along with acute appendicitis.[1][2][5][4] It may also be suspected after completing imaging of the appendix, such as a CT scan, and finding a right sided cystic mass.[2] The diagnosis is usually confirmed through biopsy or evaluation of tissues under a microscope.[1]
Last updated: 12/7/2016
The long-term outlook (prognosis) for adenocarcinoma of the appendix depends on the type and extent of the disease.[1] Overall survival of adenocarcinoma of the appendix is approximately 60% at 5 years.[2]
Last updated: 12/7/2016

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Adenocarcinoma of the appendix. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • Cancer.Net, oncologist-approved cancer information from the American Society of Clinical Oncology, has information about appendix cancer. Click on the link to view the information.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Adenocarcinoma of the appendix. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • My wife was diagnosed with low grade appendix mucinous neoplasm. I would like to know what is this tumor. See answer

  • What is mucinous adenocarcinoma of the appendix? See answer


  1. Ruoff C, Wanqing Zhi LH, Shahzad G, Gotlieb V & Wasif M. Cancers of the Appendix: Review of the Literatures. ISRN Oncology. June 26, 2011; 2011(2011):http://www.hindawi.com/journals/isrn/2011/728579/. Accessed 6/12/2015.
  2. Sarosi GA, Turnage RH.. Miscellaneous topics. In: Feldman et al, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 8th ed. Philadelphia, PA: Saunders; 2006;
  3. Epithelium. MedlinePlus. June 11, 2012; http://www.nlm.nih.gov/medlineplus/ency/article/002363.htm. Accessed 6/12/2015.
  4. Ahmed K, Hoque R, El-Tawil S, Khan MS, George ML. Adenocarcinoma of the appendix presenting as bilateral ureteric obstruction. World J Surg Oncol. 2008 Feb 21; 6(23):https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/18291037/.
  5. Hu CC, Chang JJ, Chen TC, Yen CL, Chien RN. Colonoscopic feature of primary adenocarcinoma of the appendix. Intern Med. 2008; 47(4):255-257. https://www.ncbi.nlm.nih.gov/pubmed/18277025.
  6. Appendix Cancer: Introduction. Cancer.Net. 4/2016; http://www.cancer.net/cancer-types/appendix-cancer/introduction. Accessed 12/7/2016.