Appendix cancer natural history, complications and prognosis
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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
- The majority of 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 metastases.
- Common complications of appendix cancer include:
- Acute appendicitis
- Most of appendix adenocarcinomas present with acute appendicitis.
- However, majority of carcinoids are located in the distal one third of the appendix, hence rarely present with appendicitis.
- Merely 10 percent of appendiceal carcinoids are located at the base of appendix and may tend to obstruction and appendicitis.
- Pseudomyxoma peritonei
- Carcinoid syndrome
- Acute appendicitis
- Major prognostic factors are tumor stage, tumor size, histologic as well genetic characteristics of appendiceal tumors. For TNM classification of tumors of the appendix, click here.
- 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).
- Tumor size plays a crucial role in determining prognosis.
- 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:
|Five year survival rates|
|* Shows that mucinous adenocarcinomae are less aggressive than nunmnucinous tumors|
- Poor prognostic factors for appendiceal adenocarcinoma are as follows:
- Poor prognostic factors in the presence of pseudomyxoma peritonei are as follows:
- Genetic prognostic markers of appendiceal cancers are as follows:
- Genetic studies revolutionized cancer treatment; appendix cancer is not an exception.
- Traditionally appendiceal cancers were approached the same as colorectal cancers.
- It has been shown that mutation profiles are associated with the patients’ prognosis. 
- Mutations in the TP53 significantly decrease life expectancy in patients with appendix cancer.
- Regardless of tumor grade, TP53 mutations were associated with poorer outcomes.
- Patients with GNAS mutations had a life expectancy of 10 years after diagnosis.
- Appendiceal tumors with GNAS mutations rarely develop into high-grade tumors.
- 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
- Modlin IM, Sandor A (1997). "An analysis of 8305 cases of carcinoid tumors". Cancer. 79 (4): 813–29. PMID 9024720.
- 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
- 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
- </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.
- 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.