Appendix cancer natural history, complications and prognosis: Difference between revisions
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** Spread of the tumor beyond the left lower quadrant | ** Spread of the tumor beyond the left lower quadrant | ||
** Presence of malignant cells outside of the visceral peritoneum of the appendix | ** Presence of malignant cells outside of the visceral peritoneum of the appendix | ||
* '''Poor prognostic factors in the presence of''' '''pseudomyxoma peritonei''' are as follows: | * '''Poor prognostic factors in the presence of''' '''[[pseudomyxoma peritonei]]''' are as follows: | ||
** Abdominal distension | ** Abdominal distension | ||
** Weight loss | ** Weight loss | ||
Revision as of 20:02, 18 January 2019
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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. If left untreated, the majority of patients with appendix cancer may progress to develop peritoneal carcinomatosis and metastases. Prognosis is generally excellent and good in carcinoid tumors and adenocarcinomae respectively. Prognostic factors including tumor stage, tumor size, histologic as well genetic characteristics of appendiceal tumors are discussed in details below.
Natural History, Complications, and Prognosis
Natural History
- 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.
- If left untreated, the majority of patients with appendix cancer may progress to develop peritoneal carcinomatosis and metastases
Complications
- Common complications of appendix cancer include:
Prognosis
- Major prognostic factors are tumor stage, tumor size, histologic as well genetic characteristics of appendiceal tumors.
- Prognosis is generally excellent and good in carcinoid tumors and adenocarcinomae respectively.
- The overall 5-year survival rate for adenocarcinoma of appendix is approximately 71% (see the Table below). [1]
- 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 prognosis among patients with appendical 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.
| Five year survival rates | ||
|---|---|---|
| Carcinoid tumors | ||
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| Adenocarcinoma | ||
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| * Shows that mucinous adenocarcinomae are less aggressive than nunmnucinous tumors | ||
- Poor prognostic factors for appendiceal adenocarcinoma are as follows:
- Advanced stage
- High grade
- Nonmucinous histology
- Spread of the tumor beyond the left lower quadrant
- Presence of malignant cells outside of the visceral peritoneum of the appendix
- Poor prognostic factors in the presence of pseudomyxoma peritonei are as follows:
- Abdominal distension
- Weight loss
- High histological grade
- Morphological evidence of invasion of underlying structures
- Genetic prognostic markers of appendiceal cancers are as follows:
- Genetic studies revolutionized cancer treatment; appendix cancer is not an exception.
- Traditionally appandiceal cancers were approached the same as colorectal cancers.
- Recent genetic studies demonstrated that appendiceal tumors are clearly differ from colorectal cancers. [4]=Presence of mutated TP53 and APC genes were significantly lower in appendiceal cancers compared to colorectal cancers.
- It has been shown that mutation profiles are associated with the patients’ prognosis. [5]
- Mutations in the TP53 significantly decrease life expectancy in patients with appendix cancer.
- Regardless of tumor grade, Tp 53 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.
References
- ↑ 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
|month=ignored (help) - ↑ 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) - ↑ </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.