Appendix cancer: Difference between revisions

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==Classification==
==Classification==
*Appendix cancer may be classified according to WHO classification into 4 groups:
*Appendix cancer may be classified according to WHO classification into 4 groups:<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 April 6, 2016</ref>
:*Epithelial tumors
:*Epithelial tumors
:*Non-epithelial tumors
:*Non-epithelial tumors
:*Secondary tumors
:*Secondary tumors
:*Hyperplastic (metaplastic) polyp
:*Hyperplastic (metaplastic) polyp
*The table below summarizes the different types of appendix cancer according to the WHO classification
*The table below summarizes the different types of appendix cancer according to the WHO classification.<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 April 6, 2016</ref>
{|style="border: 5px; font-size: 90%; margin: 5px; width: 300px" align=center
{|style="border: 5px; font-size: 90%; margin: 5px; width: 300px" align=center
!style="padding: 5px 5px; background: #4479BA; font-weight: bold; text-align:center;" colspan="2"|{{fontcolor|#FFF|'''WHO histological classification<br>Tumors of the appendix <br><SMALL> Adapted from WHO/IARC </SMALL>'''}}
!style="padding: 5px 5px; background: #4479BA; font-weight: bold; text-align:center;" colspan="2"|{{fontcolor|#FFF|'''WHO histological classification<br>Tumors of the appendix <br><SMALL> Adapted from WHO/IARC<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 April 6, 2016</ref> </SMALL>'''}}
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|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;"| Hyperplastic polyp
|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;"| Hyperplastic polyp
|}
|}
==Pathophysiology==
==Pathophysiology==
*The pathogenesis of appendix cancer is characterized by an initial epithelial dysplasia, followed by the formation of cystic structures and angiolymphatic invasion. Subsequently, in the advanced  stages of appendix cancer, tumor cells detach from the primary tumor mass and gain access to the peritoneal cavity.<ref name="pmid2999153">{{cite journal |vauthors=Jacobsson A, Stadler U, Glotzer MA, Kozak LP |title=Mitochondrial uncoupling protein from mouse brown fat. Molecular cloning, genetic mapping, and mRNA expression |journal=J. Biol. Chem. |volume=260 |issue=30 |pages=16250–4 |year=1985 |pmid=2999153 |doi= |url=}}</ref>
*The pathogenesis of appendix cancer is characterized by an initial epithelial dysplasia, followed by the formation of cystic structures and angiolymphatic invasion. Subsequently, in the advanced  stages of appendix cancer, tumor cells detach from the primary tumor mass and gain access to the peritoneal cavity.<ref name="pmid22084738">{{cite journal |vauthors=Ruoff C, Hanna L, Zhi W, Shahzad G, Gotlieb V, Saif MW |title=Cancers of the appendix: review of the literatures |journal=ISRN Oncol |volume=2011 |issue= |pages=728579 |year=2011 |pmid=22084738 |pmc=3200132 |doi=10.5402/2011/728579 |url=}}</ref>
*The KRAS gene mutation has been associated with the development of appendix cancer.  
*The KRAS gene mutation has been associated with the development of appendix cancer.  
*On gross pathology, findings of appendix cancer, include:<ref name="pmid22084738">{{cite journal |vauthors=Ruoff C, Hanna L, Zhi W, Shahzad G, Gotlieb V, Saif MW |title=Cancers of the appendix: review of the literatures |journal=ISRN Oncol |volume=2011 |issue= |pages=728579 |year=2011 |pmid=22084738 |pmc=3200132 |doi=10.5402/2011/728579 |url=}}</ref>
*On gross pathology, findings of appendix cancer, include:<ref name="pmid22084738">{{cite journal |vauthors=Ruoff C, Hanna L, Zhi W, Shahzad G, Gotlieb V, Saif MW |title=Cancers of the appendix: review of the literatures |journal=ISRN Oncol |volume=2011 |issue= |pages=728579 |year=2011 |pmid=22084738 |pmc=3200132 |doi=10.5402/2011/728579 |url=}}</ref>
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800px-Appendix_Carcinoid_HP_CTR.jpg | Appendiceal tumor<ref name=aaa>http://librepathology.org/wiki/index.php/Neuroendocrine_tumour_of_the_appendix</ref>
800px-Appendix_Carcinoid_HP_CTR.jpg | Appendiceal tumor<ref name=aaa>http://librepathology.org/wiki/index.php/Neuroendocrine_tumour_of_the_appendix</ref>
</gallery>
</gallery>
==Causes==
==Causes==
*Common causes of appendix cancer may include:<ref name="pmid22084738">{{cite journal |vauthors=Ruoff C, Hanna L, Zhi W, Shahzad G, Gotlieb V, Saif MW |title=Cancers of the appendix: review of the literatures |journal=ISRN Oncol |volume=2011 |issue= |pages=728579 |year=2011 |pmid=22084738 |pmc=3200132 |doi=10.5402/2011/728579 |url=}}</ref>
*Common causes of appendix cancer may include:<ref name="pmid22084738">{{cite journal |vauthors=Ruoff C, Hanna L, Zhi W, Shahzad G, Gotlieb V, Saif MW |title=Cancers of the appendix: review of the literatures |journal=ISRN Oncol |volume=2011 |issue= |pages=728579 |year=2011 |pmid=22084738 |pmc=3200132 |doi=10.5402/2011/728579 |url=}}</ref>
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==Differentiating  Appendix Cancer from Other Diseases==
==Differentiating  Appendix Cancer from Other Diseases==
* Appendix cancer must be differentiated from other diseases that cause abdominal pain, nausea, vomiting such as:
* Appendix cancer must be differentiated from other diseases that cause abdominal pain, nausea, vomiting such as:<ref name="pmid22084738">{{cite journal |vauthors=Ruoff C, Hanna L, Zhi W, Shahzad G, Gotlieb V, Saif MW |title=Cancers of the appendix: review of the literatures |journal=ISRN Oncol |volume=2011 |issue= |pages=728579 |year=2011 |pmid=22084738 |pmc=3200132 |doi=10.5402/2011/728579 |url=}}</ref>
:*[[Appendicitis]]
:*[[Appendicitis]]
:*[[Typhlitis]]
:*[[Typhlitis]]
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==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The estimated prevalence of appendix cancer is approximately 0.12 cases per 100,000 individuals in the United States.
* The estimated prevalence of appendix cancer is approximately 0.12 cases per 100,000 individuals in the United States.<ref name="pmid22302267">{{cite journal |vauthors=Turaga KK, Pappas SG, Gamblin T |title=Importance of histologic subtype in the staging of appendiceal tumors |journal=Ann. Surg. Oncol. |volume=19 |issue=5 |pages=1379–85 |year=2012 |pmid=22302267 |doi=10.1245/s10434-012-2238-1 |url=}}</ref>
* The estimated prevalence of adenocarcinoma of the appendix is 0.2 cases per 100,000 individuals worldwide.  
* The estimated prevalence of adenocarcinoma of the appendix is 0.2 cases per 100,000 individuals worldwide.<ref name="pmid22302267">{{cite journal |vauthors=Turaga KK, Pappas SG, Gamblin T |title=Importance of histologic subtype in the staging of appendiceal tumors |journal=Ann. Surg. Oncol. |volume=19 |issue=5 |pages=1379–85 |year=2012 |pmid=22302267 |doi=10.1245/s10434-012-2238-1 |url=}}</ref>
===Age===
===Age===
* The median age at diagnosis of patients with appendix cancer is 65 years.  
* The median age at diagnosis of patients with appendix cancer is 65 years.  
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===Gender===
===Gender===
*Males are more commonly affected with appendix cancer than females.
*Males are more commonly affected with appendix cancer than females.<ref name="wiki"> Appendix Cancer. Wikipedia. https://en.wikipedia.org/wiki/Appendix_cancer Accessed on April 7, 2016 </ref>


===Race===
===Race===
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==Risk Factors==
==Risk Factors==
*Common risk factors in the development of appendix cancer are family history of cancer, long-standing ulcerative colitis, and chronic inflammatory bowel diseases.
*Common risk factors in the development of appendix cancer are family history of cancer, long-standing ulcerative colitis, and chronic inflammatory bowel diseases.<ref name="pmid22302267">{{cite journal |vauthors=Turaga KK, Pappas SG, Gamblin T |title=Importance of histologic subtype in the staging of appendiceal tumors |journal=Ann. Surg. Oncol. |volume=19 |issue=5 |pages=1379–85 |year=2012 |pmid=22302267 |doi=10.1245/s10434-012-2238-1 |url=}}</ref>


== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*The majority of patients with appendix cancer may be initially asymptomatic.  
*The majority of patients with appendix cancer may be initially asymptomatic.  
*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.<ref name="wiki"> Appendix Cancer. Wikipedia. https://en.wikipedia.org/wiki/Appendix_cancer Accessed on April 7, 2016 </ref>
*Common complications of appendix cancer include perforation, peritonitis, and abscess.
*Common complications of appendix cancer include perforation, peritonitis, and abscess.
*Prognosis will depend on tumor histology. However, the overall 5 year survival rate of patients with appendix cancer is approximately 50%.
*Prognosis will depend on tumor histology. However, the overall 5 year survival rate of patients with appendix cancer is approximately 50%.<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 April 6, 2016</ref>


== Diagnosis ==
== Diagnosis ==
===Diagnostic Criteria===
===Diagnostic Criteria===
*The diagnosis of  appendix cancer is made with histopathological evaluation of the following diagnostic criteria:
*The diagnosis of  appendix cancer is made with histopathological evaluation of the following diagnostic criteria:<ref name="pmid22302267">{{cite journal |vauthors=Turaga KK, Pappas SG, Gamblin T |title=Importance of histologic subtype in the staging of appendiceal tumors |journal=Ann. Surg. Oncol. |volume=19 |issue=5 |pages=1379–85 |year=2012 |pmid=22302267 |doi=10.1245/s10434-012-2238-1 |url=}}</ref>
:*Tumor confined to the appendix
:*Tumor confined to the appendix
:*Invasion to the muscularis mucosae
:*Invasion to the muscularis mucosae
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=== Symptoms ===
=== Symptoms ===
*Symptoms of  appendix cancer may include the following:
*Symptoms of  appendix cancer may include the following:<ref name="pmid24298858">{{cite journal |vauthors=Drake FT, Flum DR |title=Improvement in the diagnosis of appendicitis |journal=Adv Surg |volume=47 |issue= |pages=299–328 |year=2013 |pmid=24298858 |pmc=4214266 |doi= |url=}}</ref>
:*Chronic [[abdominal pain]]
:*Chronic [[abdominal pain]]
::*Periumbilical
::*Periumbilical
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=== Physical Examination ===
=== Physical Examination ===
*Patients with  appendix cancer may appear normal, pale or diaphoretic.
*Patients with  appendix cancer may appear normal, pale or diaphoretic.
*Abdominal examination may be remarkable for:
*Abdominal examination may be remarkable for:<ref name="pmid24298858">{{cite journal |vauthors=Drake FT, Flum DR |title=Improvement in the diagnosis of appendicitis |journal=Adv Surg |volume=47 |issue= |pages=299–328 |year=2013 |pmid=24298858 |pmc=4214266 |doi= |url=}}</ref>
'''Palpation'''
'''Palpation'''
*The abdominal wall is very sensitive to mild palpation
*The abdominal wall is very sensitive to mild palpation
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=== Laboratory Findings ===
=== Laboratory Findings ===
*Laboratory findings consistent with the diagnosis of appendix cancer, may include:
*Laboratory findings consistent with the diagnosis of appendix cancer, may include:<ref name="pmid3696178">{{cite journal |vauthors=Moertel CG, Weiland LH, Nagorney DM, Dockerty MB |title=Carcinoid tumor of the appendix: treatment and prognosis |journal=N. Engl. J. Med. |volume=317 |issue=27 |pages=1699–701 |year=1987 |pmid=3696178 |doi=10.1056/NEJM198712313172704 |url=}}</ref>
:* [[Leukocytosis]] and a shift to the left in the segmented [[neutrophils]].
:* [[Leukocytosis]] and a shift to the left in the segmented [[neutrophils]].
:* Elevated serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA)
:* Elevated serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA)
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=== Other Diagnostic Studies ===
=== Other Diagnostic Studies ===
* Appendix cancer may also be diagnosed using the following diagnostic studies:  
* Appendix cancer may also be diagnosed using the following diagnostic studies:<ref name="pmid3696178">{{cite journal |vauthors=Moertel CG, Weiland LH, Nagorney DM, Dockerty MB |title=Carcinoid tumor of the appendix: treatment and prognosis |journal=N. Engl. J. Med. |volume=317 |issue=27 |pages=1699–701 |year=1987 |pmid=3696178 |doi=10.1056/NEJM198712313172704 |url=}}</ref>
:*[[Somatostatin]] [[scintigraphy]] with 111-indium-octreotide  
:*[[Somatostatin]] [[scintigraphy]] with 111-indium-octreotide  
:*[[Bone]] [[scintigraphy]] with 99mTc-methylene diphosphonate (99mTcMDP)  
:*[[Bone]] [[scintigraphy]] with 99mTc-methylene diphosphonate (99mTcMDP)  
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== Treatment ==
== Treatment ==
=== Medical Therapy ===  
=== Medical Therapy ===  
*The mainstay medical therapy for appendix cancer is supportive care.
*The mainstay medical therapy for appendix cancer is supportive care.<ref name="wiki"> Appendix Cancer. Wikipedia. https://en.wikipedia.org/wiki/Appendix_cancer Accessed on April 7, 2016 </ref>
*Supportive care for appendix cancer, may include:  
*Supportive care for appendix cancer, may include:<ref name="pmid3696178">{{cite journal |vauthors=Moertel CG, Weiland LH, Nagorney DM, Dockerty MB |title=Carcinoid tumor of the appendix: treatment and prognosis |journal=N. Engl. J. Med. |volume=317 |issue=27 |pages=1699–701 |year=1987 |pmid=3696178 |doi=10.1056/NEJM198712313172704 |url=}}</ref>
:*[[Somatostatin]] analogs
:*[[Somatostatin]] analogs
:*[[Interferons]]
:*[[Interferons]]
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=== Surgery ===
=== Surgery ===
*Surgery is the mainstay of therapy for appendix cancer.
*Surgery is the mainstay of therapy for appendix cancer.
*Right hemicolectomy in conjunction with chemotherapy is the most common approach to the treatment of appendix cancer.
*Right hemicolectomy in conjunction with chemotherapy is the most common approach to the treatment of appendix cancer.


=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for appendix cancer
*There are no primary preventive measures available for appendix cancer
*Once diagnosed and successfully treated, patients with  appendix cancer are followed-up every 3, 12 months.  
*Once diagnosed and successfully treated, patients with  appendix cancer are followed-up every 3, 12 months.  
*Follow-up testing for appendix cancer includes physical examination, medical history, imaging studies, chromogranin testing, and laboratory studies (eg. tumoral biomarkers)  
*Follow-up testing for appendix cancer includes physical examination, medical history, imaging studies, chromogranin testing, and laboratory studies (eg. tumoral biomarkers).<ref name="pmid3696178">{{cite journal |vauthors=Moertel CG, Weiland LH, Nagorney DM, Dockerty MB |title=Carcinoid tumor of the appendix: treatment and prognosis |journal=N. Engl. J. Med. |volume=317 |issue=27 |pages=1699–701 |year=1987 |pmid=3696178 |doi=10.1056/NEJM198712313172704 |url=}}</ref>


==References==
==References==

Revision as of 20:09, 6 April 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Tumors of the appendix; Appendiceal cancer; Cancer of the appendix; Neoplasms of the appendix

Overview

Appendix cancer (also known as appendiceal cancer) is a rare malignancy of the vermiform appendix. The most common type of appendix cancer is carcinoid tumor. Appendix cancers often present with peritoneal seeding, resulting in peritoneal carcinomatosis or pseudomyxoma peritonei.[1] Appendix cancer may be classified according to WHO classification into 4 groups: epithelial tumors, non-epithelial tumors, secondary tumors, and hyperplastic (metaplastic) polyp. Common causes of appendix cancer may include hereditary syndromes (eg. familial adenomatous polyposis, hereditary non-polyposis colorectal cancer) and chronic inflammatory diseases (eg. ulcerative colitis, Crohn's disease). The estimated prevalence of appendix cancer is approximately 0.12 cases per 100,000 individuals in the United States. On gross pathology, well-demarcated mass, between 1-5 cm, gray or yellowish color are characteristic findings of appendix cancer. Early clinical features include periodical unspecific abdominal pain, bloating, and nausea. Enhanced CT is the imaging modality of choice for appendix cancer. On CT, appendix cancer is characterized by the following findings: soft tissue thickening, wall irregularity, presence of pseudomyxoma peritonei, calcification, and internal septations. Prognosis is will depend on tumor histology.[2] However, the overall 5-survival rate of patients with appendix cancer is approximately 51%. Surgery is the mainstay of therapy for appendix cancer. Right hemicolectomy in conjunction with chemotherapy is the most common approach to the treatment of appendix cancer. Once diagnosed and successfully treated, patients with appendix cancer are followed-up every 3, 12 months. Follow-up testing for appendix cancer includes physical examination, medical history, imaging studies, chromogranin testing, and laboratory studies (eg. tumoral biomarkers).

Historical Perspective

  • Appendix cancer was first described by Gagne, a French Physician, in 1969.[3]

Classification

  • Appendix cancer may be classified according to WHO classification into 4 groups:[4]
  • Epithelial tumors
  • Non-epithelial tumors
  • Secondary tumors
  • Hyperplastic (metaplastic) polyp
  • The table below summarizes the different types of appendix cancer according to the WHO classification.[4]
WHO histological classification
Tumors of the appendix
Adapted from WHO/IARC[4]
Epithelial tumors
  • Adenoma
  • Tubular
  • Villous
  • Tubulovillous
  • Serrated
  • Carcinoma
  • Adenocarcinoma
  • Mucinous adenocarcinoma
  • Signet-ring cell carcinoma
  • Small cell carcinoma
  • Undifferentiated carcinoma
  • Carcinoid (well differentiated endocrine neoplasm)
  • Tubular carcinoid
  • Goblet cell carcinoid (mucinous carcinoid)
  • Mixed carcinoid-adenocarcinoma
  • Others
Non-epithelial tumors
  • Neuroma
  • Lipoma
  • Leiomyoma
  • Gastrointestinal stromal tumor
  • Leiomyosarcoma
  • Kaposi sarcoma
  • Others
Secondary tumors
  • Metastasis (eg. Primary of urogenital tract, breast, lung)
Hyperplastic polyp

Pathophysiology

  • The pathogenesis of appendix cancer is characterized by an initial epithelial dysplasia, followed by the formation of cystic structures and angiolymphatic invasion. Subsequently, in the advanced stages of appendix cancer, tumor cells detach from the primary tumor mass and gain access to the peritoneal cavity.[5]
  • The KRAS gene mutation has been associated with the development of appendix cancer.
  • On gross pathology, findings of appendix cancer, include:[5]
  • Well-demarcated mass
  • Average size between 1 and 5cm
  • Gray or yellowish color
  • Deformed appendix
  • On microscopic histopathological analysis findings will depend on the subtype of appendicular cancer.
  • Common histopathological findings, may include:[5]
  • Cystic structures
  • Angiolymphatic invasion
  • The images below demonstrate different histopathological findings of appendix cancer.

Causes

  • Common causes of appendix cancer may include:[5]
  • Chronic inflammatory diseases, such as:
  • Ulcerative colitis
  • Crohn's disease
  • Hereditary syndromes, such as:
  • Familial adenomatous polyposis
  • Hereditary non-polyposis colorectal cancer

Differentiating Appendix Cancer from Other Diseases

  • Appendix cancer must be differentiated from other diseases that cause abdominal pain, nausea, vomiting such as:[5]

Epidemiology and Demographics

  • The estimated prevalence of appendix cancer is approximately 0.12 cases per 100,000 individuals in the United States.[1]
  • The estimated prevalence of adenocarcinoma of the appendix is 0.2 cases per 100,000 individuals worldwide.[1]

Age

  • The median age at diagnosis of patients with appendix cancer is 65 years.
  • Appendix cancer is more commonly observed among patients aged 50 to 60 years old.
  • Appendix cancer is more commonly observed among adults and elderly patients.

Gender

  • Males are more commonly affected with appendix cancer than females.[7]

Race

  • There is no racial predilection for appendix cancer.

Risk Factors

  • Common risk factors in the development of appendix cancer are family history of cancer, long-standing ulcerative colitis, and chronic inflammatory bowel diseases.[1]

Natural History, Complications and Prognosis

  • The majority of patients with appendix cancer may be initially asymptomatic.
  • 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.[7]
  • Common complications of appendix cancer include perforation, peritonitis, and abscess.
  • Prognosis will depend on tumor histology. However, the overall 5 year survival rate of patients with appendix cancer is approximately 50%.[4]

Diagnosis

Diagnostic Criteria

  • The diagnosis of appendix cancer is made with histopathological evaluation of the following diagnostic criteria:[1]
  • Tumor confined to the appendix
  • Invasion to the muscularis mucosae
  • Presence of metastases
  • Spread to the peritoneal cavity

Symptoms

  • Symptoms of appendix cancer may include the following:[8]
  • Periumbilical
  • Right lower quadrant

Physical Examination

  • Patients with appendix cancer may appear normal, pale or diaphoretic.
  • Abdominal examination may be remarkable for:[8]

Palpation

Rectal examination'

Laboratory Findings

  • Laboratory findings consistent with the diagnosis of appendix cancer, may include:[9]
  • Leukocytosis and a shift to the left in the segmented neutrophils.
  • Elevated serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA)
  • Elevated c-reactive protein (CRP)

Imaging Findings

  • Enhanced CT is the imaging modality of choice for appendix cancer.
  • On CT, appendix cancer is characterized by the following findings:[2]
  • Soft tissue thickening
  • Wall irregularity
  • Presence of pseudomyxoma peritonei
  • Calcification
  • Internal septations
  • Periappendiceal fat stranding and intraperitoneal free fluid is nonspecific.

Other Diagnostic Studies

  • Appendix cancer may also be diagnosed using the following diagnostic studies:[9]

Treatment

Medical Therapy

  • The mainstay medical therapy for appendix cancer is supportive care.[7]
  • Supportive care for appendix cancer, may include:[9]

Surgery

  • Surgery is the mainstay of therapy for appendix cancer.
  • Right hemicolectomy in conjunction with chemotherapy is the most common approach to the treatment of appendix cancer.

Prevention

  • There are no primary preventive measures available for appendix cancer
  • Once diagnosed and successfully treated, patients with appendix cancer are followed-up every 3, 12 months.
  • Follow-up testing for appendix cancer includes physical examination, medical history, imaging studies, chromogranin testing, and laboratory studies (eg. tumoral biomarkers).[9]

References

  1. 1.0 1.1 1.2 1.3 1.4 Turaga KK, Pappas SG, Gamblin T (2012). "Importance of histologic subtype in the staging of appendiceal tumors". Ann. Surg. Oncol. 19 (5): 1379–85. doi:10.1245/s10434-012-2238-1. PMID 22302267.
  2. 2.0 2.1 Hoeffel C, Crema MD, Belkacem A, Azizi L, Lewin M, Arrivé L, Tubiana JM (2006). "Multi-detector row CT: spectrum of diseases involving the ileocecal area". Radiographics. 26 (5): 1373–90. doi:10.1148/rg.265045191. PMID 16973770.
  3. Gagné F, Fortin P, Dufour V, Delage C (1969). "[Tumors of the appendix associating histologic features of carcinoid and adenocarcinoma]". Ann Anat Pathol (Paris) (in French). 14 (4): 393–406. PMID 5378353.
  4. 4.0 4.1 4.2 4.3 Chapter 5: Tumours of the Appendix - IARC. https://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb2/bb2-chap5.pdf Accessed on April 6, 2016
  5. 5.0 5.1 5.2 5.3 5.4 Ruoff C, Hanna L, Zhi W, Shahzad G, Gotlieb V, Saif MW (2011). "Cancers of the appendix: review of the literatures". ISRN Oncol. 2011: 728579. doi:10.5402/2011/728579. PMC 3200132. PMID 22084738.
  6. 6.0 6.1 6.2 6.3 6.4 http://librepathology.org/wiki/index.php/Neuroendocrine_tumour_of_the_appendix
  7. 7.0 7.1 7.2 Appendix Cancer. Wikipedia. https://en.wikipedia.org/wiki/Appendix_cancer Accessed on April 7, 2016
  8. 8.0 8.1 Drake FT, Flum DR (2013). "Improvement in the diagnosis of appendicitis". Adv Surg. 47: 299–328. PMC 4214266. PMID 24298858.
  9. 9.0 9.1 9.2 9.3 Moertel CG, Weiland LH, Nagorney DM, Dockerty MB (1987). "Carcinoid tumor of the appendix: treatment and prognosis". N. Engl. J. Med. 317 (27): 1699–701. doi:10.1056/NEJM198712313172704. PMID 3696178.