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==Overview== | ==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 | Because of the location and size of [[Vermiform appendix|appendix]], most of the [[Appendix cancer (patient information)|patients with appendix cancer]] may be initially [[asymptomatic]]. Early clinical features might include periodical unspecific [[abdominal pain]], [[bloating]], and [[Nausea and vomiting|nausea]]. Most of [[Vermiform appendix|appendix]] [[cancer]] cases are discovered after [[Surgery|surgical]] or [[Histology|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]]. [[Vermiform appendix|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 Tumor|carcinoid]] [[Tumor|tumors]] and [[adenocarcinoma]] respectively. [[Prognosis|Prognostic factors]] including tumor stage, [[tumor]] size, [[Histology|histologic]] as well [[Genetics|genetic]] characteristics of appendiceal tumors were discussed in details. | ||
==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
===Natural History=== | ===Natural History=== | ||
*The majority of patients with appendix cancer may be initially asymptomatic. | *The majority of patients with [[Vermiform appendix|appendix]] [[cancer]] may be initially [[asymptomatic]]. | ||
*Early clinical features might include periodical unspecific abdominal pain, bloating, and nausea. | *Early clinical features might include periodical unspecific [[abdominal pain]], [[bloating]], and [[Nausea and vomiting|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. | *Most of [[Vermiform appendix|appendix]] [[cancer]] cases are discovered after [[Surgery|surgical]] or [[Histology|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. | *Around one percent of all [[appendectomy]] specimens are [[malignant]]. | ||
*Appendix cancer account for 0.5 percent of all intestinal | *Appendix cancer account for 0.5 percent of all intestinal [[neoplasm]]<nowiki/>s. | ||
*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 [[Metastasis|metastases]]. | ||
===Complications=== | ===Complications=== | ||
*Common complications of appendix cancer include: | *Common complications of [[Vermiform appendix|appendix]] [[cancer]] include: | ||
**[[Appendicitis|Acute appendicitis]] | **[[Appendicitis|Acute appendicitis]] | ||
*** Most of appendix [[adenocarcinoma]]<nowiki/>s present with [[Appendicitis|acute appendicitis]]. | |||
***However, majority of <nowiki/>[[Carcinoid Tumor|carcinoid]]<nowiki/>s are located in the distal one third of the [[Vermiform appendix|appendix]], hence rarely present with [[appendicitis]]. | |||
***Merely 10 percent of appendiceal [[Carcinoid Tumor|carcinoid]]<nowiki/>s are located at the base of [[Vermiform appendix|appendix]] and may tend to obstruction and [[appendicitis]]. | |||
**[[Pseudomyxoma peritonei]] | **[[Pseudomyxoma peritonei]] | ||
**Metastasis | **[[Metastasis]] | ||
**[[Carcinoid syndrome]] | **[[Carcinoid syndrome]] | ||
***More commonly seen in [[midgut]] ([[Vermiform appendix|appendix]] and [[Small intestine|small bowel]]) [[Carcinoid Tumor|carcinoid]]<nowiki/>s. | |||
***Almost always (90%) seen in [[Metastasis|metastatic]] disease, typically [[liver]] is the involved organ. | |||
===Prognosis=== | ===Prognosis=== | ||
* | * | ||
* | * | ||
*Major prognostic factors are tumor stage, tumor size, histologic as well genetic characteristics of appendiceal | *Major [[Prognosis|prognostic]] factors are [[Cancer staging|tumor stage]], [[tumor]] size, [[Histology|histologic]] as well [[Genetics|genetic]] characteristics of appendiceal [[tumor]]<nowiki/>s. For [[TNM|TNM classification]] of [[tumor|t]]<nowiki/>[[tumor|umor]]<nowiki/>s of the [[Vermiform appendix|appendix]], [[Appendix cancer diagnostic study of choice|click here]].<ref name="WHO">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</ref> | ||
*[[Prognosis]] is generally excellent and good in [[Carcinoid Tumor|carcinoid tumors]] and [[adenocarcinoma]]<nowiki/>s respectively. | |||
*The overall 5-year survival rate for adenocarcinoma of appendix is approximately 71% (see the Table below).<ref name="pmid9024720">{{cite journal| author=Modlin IM, Sandor A| title=An analysis of 8305 cases of carcinoid tumors. | journal=Cancer | year= 1997 | volume= 79 | issue= 4 | pages= 813-29 | pmid=9024720 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9024720 }}</ref> | |||
*Prognosis is generally excellent and good in carcinoid tumors and | |||
*The overall 5-year survival rate for adenocarcinoma of appendix is approximately 71% (see the Table below). <ref name="pmid9024720">{{cite journal| author=Modlin IM, Sandor A| title=An analysis of 8305 cases of carcinoid tumors. | journal=Cancer | year= 1997 | volume= 79 | issue= 4 | pages= 813-29 | pmid=9024720 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9024720 }}</ref> | |||
*'''Tumor size''' plays a crucial role in determining prognosis. | *'''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.<ref name=":0">{{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 name=":0">{{Cite journal | ||
| Line 140: | Line 60: | ||
| pmid = 15213627 | | pmid = 15213627 | ||
}}</ref> | }}</ref> | ||
*The prognosis varies with the histology of tumor. | *The prognosis varies with the [[histology]] of tumor. | ||
*Carcinoid tumors are associated with a better prognosis | *[[Carcinoid Tumor|Carcinoid tumors]] are associated with a better [[prognosis]] among patients with appendiceal cancer. | ||
*Localized carcinoid tumors are associated with the most favorable prognosis. | *Localized [[Carcinoid Tumor|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. | *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. | *Goblet cell carcinoids are generally more aggressive than other [[Carcinoid Tumor|carcinoid tumors]]. | ||
*According to | *According to surveillance, [[epidemiology]] and end results (SEER) database of the [[National Cancer Institute|national cancer institute]] five-year appendiceal [[Carcinoid Tumor|carcinoid]] [[survival rate]]<nowiki/>s were as follows: | ||
:* '''Tumor size <3 cm''' without regional nodal or distant | :* '''Tumor size <3 cm''' without regional nodal or distant [[Metastasis|metastas]]<nowiki/>es: 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 | :* '''Tumor size between 2 and 3 cm''' plus regional node [[Metastasis|metastases]] / tumor size ≥3 cm with or without regional nodal or distant [[Metastasis|metastases]]: 78 percent | ||
:* '''Distant metastasis:''' 32 percent | :* '''Distant metastasis:''' 32 percent | ||
{| style="border: 5px; font-size: 90%; margin: 5px; width: 300px" align="center" | {| class="wikitable" style="border: 5px; font-size: 90%; margin: 5px; width: 300px" align="center" | ||
! | ! style="padding: 5px 5px; background: #4479BA; font-weight: bold; text-align:center;" |{{fontcolor|#FFF|Five year survival rates}} | ||
|+ | |+ | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" | Carcinoid tumors | | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" |Carcinoid tumors | ||
|- | |- | ||
| | | | ||
| Line 173: | Line 92: | ||
:*Distant metastasis 51%* | :*Distant metastasis 51%* | ||
|- | |- | ||
|''* Shows that mucinous adenocarcinomae are less aggressive'' ''than nunmnucinous tumors'' | |<small>''* Shows that mucinous adenocarcinomae are less aggressive'' ''than nunmnucinous tumors''</small> | ||
|} | |} | ||
* '''Poor prognostic factors''' for '''appendiceal adenocarcinoma''' are as follows: | * '''Poor [[Prognosis|prognostic]] factors''' for '''appendiceal [[adenocarcinoma]]''' are as follows: | ||
** Advanced stage | ** Advanced [[Cancer staging|stage]] | ||
**High grade | **High grade | ||
** | ** Non-mucinous [[histology]] | ||
** 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]] | ||
** High histological grade | ** High histological grade | ||
** Morphological evidence of invasion of underlying structures | ** Morphological evidence of invasion of underlying structures | ||
*'''Genetic prognostic markers of appendiceal cancers''' are as follows: | *'''[[Genetics|Genetic]] [[Prognosis|prognostic]] markers of appendiceal cancers''' are as follows: | ||
**Genetic studies revolutionized cancer treatment; appendix cancer is not an exception. | **[[Genetics|Genetic]] studies revolutionized [[cancer]] treatment; [[Vermiform appendix|appendix]] [[cancer]] is not an exception. | ||
**Traditionally | **Traditionally appendiceal cancers were approached the same as [[colorectal cancer]]<nowiki/>s. | ||
***Recent genetic studies demonstrated that appendiceal tumors are clearly differ from colorectal | ***Recent [[Genetics|genetic]] studies demonstrated that appendiceal tumors are clearly differ from [[colorectal cancer]]<nowiki/>s. <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 | ***Presence of mutated [[TP53]] and [[APC]] genes were significantly lower in appendiceal cancers compared to [[colorectal cancer]]<nowiki/>s. | ||
** It has been shown that mutation profiles are associated with the patients’ prognosis. <ref name="pmid26821970">{{cite journal| author=Levine EA, Votanopoulos KI, Qasem SA, Philip J, Cummins KA, Chou JW et al.| title=Prognostic Molecular Subtypes of Low-Grade Cancer of the Appendix. | journal=J Am Coll Surg | year= 2016 | volume= 222 | issue= 4 | pages= 493-503 | pmid=26821970 | doi=10.1016/j.jamcollsurg.2015.12.012 | pmc=4808611 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26821970 }} </ref> | ** It has been shown that mutation profiles are associated with the patients’ [[prognosis]]. <ref name="pmid26821970">{{cite journal| author=Levine EA, Votanopoulos KI, Qasem SA, Philip J, Cummins KA, Chou JW et al.| title=Prognostic Molecular Subtypes of Low-Grade Cancer of the Appendix. | journal=J Am Coll Surg | year= 2016 | volume= 222 | issue= 4 | pages= 493-503 | pmid=26821970 | doi=10.1016/j.jamcollsurg.2015.12.012 | pmc=4808611 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26821970 }} </ref> | ||
*** | *** [[Mutation]]<nowiki/>s in the [[TP53]] significantly decrease life expectancy in patients with appendix cancer. | ||
*** Regardless of tumor grade, | *** Regardless of tumor grade, [[TP53]] mutations were associated with poorer outcomes. | ||
*** Patients with GNAS | *** Patients with [[GNAS complex locus|GNAS]] [[mutation]]<nowiki/>s had a [[life expectancy]] of 10 years after diagnosis. | ||
*** Appendiceal | *** Appendiceal [[tumor]]<nowiki/>s with [[GNAS complex locus|GNAS]] [[mutation]]<nowiki/>s rarely develop into high-grade tumors. | ||
==References== | ==References== | ||
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{{WH}} | {{WH}} | ||
{{WS}} | {{WS}} | ||
[[Category: | [[Category:Surgery]] | ||
[[Category:Medicine]] | |||
[[Category:Emergency medicine]] | |||
[[Category:Oncology]] | |||
[[Category:Up-To-Date]] | |||
[[Category:Genetics]] | |||
[[Category:Radiology]] | |||
Latest revision as of 17:48, 22 February 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. 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
- 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.
Complications
- 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
- Metastasis
- Carcinoid syndrome
- More commonly seen in midgut (appendix and small bowel) carcinoids.
- Almost always (90%) seen in metastatic disease, typically liver is the involved organ.
- Acute appendicitis
Prognosis
- 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.[1]
- 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.
- 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 |
|
| Adenocarcinoma |
|
| * Shows that mucinous adenocarcinomae are less aggressive than nunmnucinous tumors |
- Poor prognostic factors for appendiceal adenocarcinoma are as follows:
- Advanced stage
- High grade
- Non-mucinous 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 appendiceal cancers were approached the same as colorectal cancers.
- Recent genetic studies demonstrated that appendiceal tumors are clearly differ from colorectal cancers. [5]
- 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. [6]
- 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.
References
- ↑ 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
|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.