Appendix cancer natural history, complications and prognosis: Difference between revisions
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*The majority of patients with appendix cancer may be initially asymptomatic. | *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 | * 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. | *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. | *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. | |||
*Tumor size plays a crucial role in determining prognosis. | *Tumor size plays a crucial role in determining prognosis. | ||
*Most of appendiceal tumors are less than 2 cm in size and have a quite favorable prognosis since they barely metastasize.<ref>{{Cite journal | *Most of the appendiceal tumors are less than 2 cm in size and have a quite favorable prognosis since they barely metastasize.<ref>{{Cite journal | ||
| author = [[Irvin M. Modlin]], [[Kevin D. Lye]] & [[Mark Kidd]] | | author = [[Irvin M. Modlin]], [[Kevin D. Lye]] & [[Mark Kidd]] | ||
| title = A 5-decade analysis of 13,715 carcinoid tumors | | title = A 5-decade analysis of 13,715 carcinoid tumors | ||
| Line 98: | Line 97: | ||
** High histological grade | ** High histological grade | ||
** Morphological evidence of invasion of underlying structures | ** Morphological evidence of invasion of underlying structures | ||
?????????? | ?????????? | ||
*Genetic studies revolutionized cancer treatment, appendix cancer is not an exception. | *Genetic studies revolutionized cancer treatment, appendix cancer is not an exception. | ||
* Traditionally appandiceal cancers were approached the same as colorectal cancers. | * Traditionally appandiceal cancers were approached the same as colorectal cancers. | ||
* Recent genetic studies demonstrated that appendiceal tumors are clearly differ from colorectal cancers.<ref name="pmid22342786">{{cite journal| author=Levine EA, Blazer DG, Kim MK, Shen P, Stewart JH, Guy C et al.| title=Gene expression profiling of peritoneal metastases from appendiceal and colon cancer demonstrates unique biologic signatures and predicts patient outcomes. | journal=J Am Coll Surg | year= 2012 | volume= 214 | issue= 4 | pages= 599-606; discussion 606-7 | pmid=22342786 | doi=10.1016/j.jamcollsurg.2011.12.028 | pmc=3768122 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22342786 }} </ref> | * Recent genetic studies demonstrated that appendiceal tumors are clearly differ from colorectal cancers.<nowiki><ref name="pmid22342786"></nowiki>{{cite journal| author=Levine EA, Blazer DG, Kim MK, Shen P, Stewart JH, Guy C et al.| title=Gene expression profiling of peritoneal metastases from appendiceal and colon cancer demonstrates unique biologic signatures and predicts patient outcomes. | journal=J Am Coll Surg | year= 2012 | volume= 214 | issue= 4 | pages= 599-606; discussion 606-7 | pmid=22342786 | doi=10.1016/j.jamcollsurg.2011.12.028 | pmc=3768122 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22342786 }} </ref> | ||
* Presence of mutated TP53 and APC genes were significantly lower in appendiceal cancers compared to colorectal cancers. | * Presence of mutated TP53 and APC genes were significantly lower in appendiceal cancers compared to colorectal cancers. | ||
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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.
- 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.[1][2]A poorer survival has been reported for the black patients with carcinoid tumors. [2]
- The overall 5-year survival rate for adenocarcinoma of appendix is approximately 71% (see the Table below). [3]
- 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. [4]
- 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
- ↑ 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) - ↑ 2.0 2.1 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) - ↑ Modlin IM, Sandor A (1997). "An analysis of 8305 cases of carcinoid tumors". Cancer. 79 (4): 813–29. PMID 9024720.
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:
- Acute appendicitis
- Pseudomyxoma pritonei
- ?????????
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.
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 - 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 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.<ref name="pmid22342786">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.