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
- The majority of patients with appendix cancer may be initially asymptomatic. 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. Early clinical features include periodical unspecific abdominal pain, bloating, and nausea. 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. 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. Most of the appendiceal tumors are less than 2 cm in size and have a quite favorable prognosis since they barely metastasize.[2][3] Other prognostic factors such as histologic and genetic characteristics of appendiceal tumors are discussed in details below.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
- The symptoms of (disease name) typically develop ___ years after exposure to ___.
- If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
Complications
- Common complications of appendix cancer include:
Prognosis
- Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [--]%.
- Depending on the extent of the [tumor/disease progression] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
- The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
- [Subtype of disease/malignancy] is associated with the most favorable prognosis.
- The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.
- 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.
- Most of the appendiceal tumors are less than 2 cm in size and have a quite favorable prognosis since they barely metastasize.[2][3]
- Other prognostic factors such as histologic and genetic characteristics of appendiceal tumors are discussed in details below.
| 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
- ↑ 1.0 1.1 Modlin IM, Sandor A (1997). "An analysis of 8305 cases of carcinoid tumors". Cancer. 79 (4): 813–29. PMID 9024720.
- ↑ 2.0 2.1 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) - ↑ 3.0 3.1 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.