Appendix cancer natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]


Overview

Because of the location and size of appendix, most of the patients with appendix cancer may be initially asymptomatic. Early clinical features might include periodical unspecific abdominal pain, bloating, and nausea. Most of appendix cancer cases are discovered after surgical or histological evaluation of a patient with acute appendicitis, or are an accidental finding in imaging studies for the other reasons. Around one percent of all appendectomy specimens are malignant. Appendix cancer account for 0.5 percent of all intestinal neoplasms. If left untreated, the majority of patients with appendix cancer may progress to develop peritoneal carcinomatosis and metastasis. Prognosis is generally excellent and good in carcinoid tumors and adenocarcinoma respectively. Prognostic factors including tumor stage, tumor size, histologic as well genetic characteristics of appendiceal tumors were discussed in details.

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

  • Prognosis is generally excellent and good in carcinoid tumors and adenocarcinomas respectively.
  • The overall 5-year survival rate for adenocarcinoma of appendix is approximately 71% (see the Table below).[2]
  • Tumor size plays a crucial role in determining prognosis.
    • Most of the appendiceal tumors are less than 2 cm in size and have a quite favorable prognosis since they barely metastasize.[3][4]
  • The prognosis varies with the histology of tumor.
  • Carcinoid tumors are associated with a better prognosis among patients with appendiceal cancer.
  • Localized carcinoid tumors are associated with the most favorable prognosis.
  • The presence of tubular pathology is also associated with a particularly good prognosis among patients with appendix adenocarcinoma.
  • Goblet cell carcinoids are generally more aggressive than other carcinoid tumors.
  • According to surveillance, epidemiology and end results (SEER) database of the national cancer institute five-year appendiceal carcinoid survival rates were as follows:
  • Tumor size <3 cm without regional nodal or distant metastases: 100 percent
  • Tumor size between 2 and 3 cm plus regional node metastases / tumor size ≥3 cm with or without regional nodal or distant metastases: 78 percent
  • Distant metastasis: 32 percent
Five year survival rates
Carcinoid tumors
  • localized disease 94%
  • Regional disease 85%
  • Distant metastases 34%
  • Goblet cell 12.5%
  • Tubular tumors are benign clinically
Adenocarcinoma
Nonmucinus
  • Localized 95%
  • Distant metastasis 0%*
Mucinous adenocarcinoma
  • localized 80%
  • Distant metastasis 51%*
* Shows that mucinous adenocarcinomae are less aggressive than nunmnucinous tumors

References

  1. Chapter 5: Tumours of the Appendix - IARC. https://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb2/bb2-chap5.pdf Accessed on January 15, 2019
  2. Modlin IM, Sandor A (1997). "An analysis of 8305 cases of carcinoid tumors". Cancer. 79 (4): 813–29. PMID 9024720.
  3. Irvin M. Modlin, Kevin D. Lye & Mark Kidd (2003). "A 5-decade analysis of 13,715 carcinoid tumors". Cancer. 97 (4): 934–959. doi:10.1002/cncr.11105. PMID 12569593. Unknown parameter |month= ignored (help)
  4. A poorer survival has been reported for the black patients with carcinoid tumors. <ref name=":0">Melinda A. Maggard, Jessica B. O'Connell & Clifford Y. Ko (2004). "Updated population-based review of carcinoid tumors". Annals of surgery. 240 (1): 117–122. PMID 15213627. Unknown parameter |month= ignored (help)
  5. </nowiki>Levine EA, Blazer DG, Kim MK, Shen P, Stewart JH, Guy C; et al. (2012). "Gene expression profiling of peritoneal metastases from appendiceal and colon cancer demonstrates unique biologic signatures and predicts patient outcomes". J Am Coll Surg. 214 (4): 599–606, discussion 606-7. doi:10.1016/j.jamcollsurg.2011.12.028. PMC 3768122. PMID 22342786.
  6. Levine EA, Votanopoulos KI, Qasem SA, Philip J, Cummins KA, Chou JW; et al. (2016). "Prognostic Molecular Subtypes of Low-Grade Cancer of the Appendix". J Am Coll Surg. 222 (4): 493–503. doi:10.1016/j.jamcollsurg.2015.12.012. PMC 4808611. PMID 26821970.