August Is Appendix Cancer Awareness Month

TON - August 2023 Vol 16, No 4

Cancers and tumors of the appendix are extremely rare, with an estimated incidence of 0.15 to 0.9 per 100,000 people. The average age of onset is between 50 and 55 years, and the disease appears to affect men and women equally.1 Evidence suggests that the incidence of appendix cancer has increased over the past 2 decades.2

There are different types of tumors that can originate in the appendix, including neuroendocrine tumors, appendiceal mucoceles, colonic-type adenocarcinoma, signet-ring cell adenocarcinoma, goblet cell carcinomas/adenoneuroendocrines, and paraganglioma.2

  • Appendix cancer is frequently misdiagnosed because patients may present with relatively common symptoms, such as pain in the abdomen or pelvis area, increased abdominal girth, changes in bowel function, bloating, hernia, fluid in the abdomen, or infertility, which are associated with other types of conditions.1,2
  • For many patients, appendix cancer is discovered during an unrelated surgical procedure, such as an appendectomy due to appendicitis. In some cases, imaging tests, such as X-rays or computed tomography scans, reveal existing tumors.2,3
  • Treatment approaches for appendix cancer vary depending on the type and stage of the disease, as well as specific patient-related factors. For low-grade mucinous neoplasm and low-grade mucinous adenocarcinomas confined to the abdominal cavity, the most successful treatment is a combination therapy that consists of specialized cytoreductive (tumor removal) surgery to remove all of the visible tumors and affected tissues, followed by localized application of heated intraperitoneal chemotherapy to eliminate residual microscopic disease.4
  • The Appendix Cancer Pseudomyxoma Peritonei (ACPMP) Research Foundation provides a wide range of resources to support patients with appendix cancer and pseudomyxoma peritonei (PMP) and their loved ones, including personal one-on-one guidance. A patient guide is available on the foundation’s website, providing links to resources and information about critical next steps following diagnosis. The website also features a specialist map identifying physicians by state who have experience with this rare type of cancer.5
  • Additional features found on the ACPMP website include a resource page with links to educational materials, a video library of past educational events, and a Facebook group with more than 4800 members. Visitors to the website will also have access to a Patient Advisory Council comprised of patients, caregivers, and advocates who provide perspectives on their experiences.5
  • The ACPMP foundation hosts numerous educational events each year. Virtual webinars feature researchers and physicians who discuss new developments in the diagnosis and treatment of appendix cancer and PMP. The foundation also partners with several institutions that treat appendix cancer and PMP to host regional patient-physician symposiums.5


  1. The Appendix Cancer Pseudomyxoma Peritonei Research Foundation. Appendix cancer & pseudomyxoma peritonei fact sheet. Updated November 2022. Accessed July 20, 2023.
  2. Appendix cancer. Updated February 2023. Accessed July 20, 2023.
  3. Cleveland Clinic. Appendix cancer. Updated November 30, 2023. Accessed July 31, 2023.
  4. The Appendix Cancer Pseudomyxoma Peritonei Research Foundation. About appendix cancer. Accessed July 31, 2023.
  5. The Appendix Cancer Pseudomyxoma Peritonei Research Foundation. Home. Accessed July 31, 2023.

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