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| __NOTOC__ | | __NOTOC__ |
| {{SI}} | | {{Appendix cancer}} |
| {{CMG}} {{AE}} {{MV}}
| | '''For patient information, click [[Appendix cancer (patient information)|here]]''' |
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| {{SK}} Tumors of the appendix; Appendiceal cancer; Cancer of the appendix; Neoplasms of the appendix
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| ==Overview==
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| '''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.<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> Appendix cancer may be classified according to WHO classification into 4 groups:
| | {{CMG}}; {{AE}} {{Soroush}} |
| 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.
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| ==Historical Perspective==
| | {{SK}}Appendix malignancy, Appendix tumor, Appendiceal cancer, Appendiceal malignancy, Carcinoid tumor of appendix, Adenocarcinoma of appendix, Goblet cell tumor of appendix, |
| *Appendix cancer was first described by Gagne, a French Physician, in 1969.<ref name="pmid5378353">{{cite journal |vauthors=Gagné F, Fortin P, Dufour V, Delage C |title=[Tumors of the appendix associating histologic features of carcinoid and adenocarcinoma] |language=French |journal=Ann Anat Pathol (Paris) |volume=14 |issue=4 |pages=393–406 |year=1969 |pmid=5378353 |doi= |url=}}</ref>
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| ==Classification== | | ==[[Appendix cancer overview|Overview]]== |
| *Appendix cancer may be classified according to WHO classification into 4 groups:
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| :*Epithelial tumors
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| :*Non-epithelial tumors
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| :*Secondary tumors
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| :*Hyperplastic (metaplastic) polyp
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| *The table below summarizes the different types of appendix cancer according to the WHO classification
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| {|style="border: 5px; font-size: 90%; margin: 5px; width: 800px" align=center
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| !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>'''}}
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| |valign=top|
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| | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;"| Epithelial tumors
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| *Adenoma
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| :*Tubular
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| :*Villous
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| :*Tubulovillous
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| :*Serrated
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| *Carcinoma
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| :*Adenocarcinoma
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| :*Mucinous adenocarcinoma
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| :*Signet-ring cell carcinoma
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| :*Small cell carcinoma
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| :*Undifferentiated carcinoma
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| *Carcinoid (well differentiated endocrine neoplasm)
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| *Tubular carcinoid
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| *Goblet cell carcinoid (mucinous carcinoid)
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| *Mixed carcinoid-adenocarcinoma
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| *Others
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| |-
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| | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" | Non-epithelial tumors
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| *Neuroma
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| *Lipoma
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| *Leiomyoma
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| *Gastrointestinal stromal tumor
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| *Leiomyosarcoma
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| *Kaposi sarcoma
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| *Others
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| |-
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| |style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;"| Secondary tumors
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| |-
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| *Metastasis (eg. Primary of urogenital tract, breast, lung)
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| |-
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| |style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;"| Hyperplastic polyp
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| |}
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| ==Pathophysiology== | | ==[[Appendix cancer historical perspective|Historical Perspective]]== |
| *The pathogenesis of appendix cancer is characterized by an initial epithelial dysplasia.
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| *The KRAS gene mutation has been associated with the development of appendix cancer.
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| *On gross pathology, well-demarcated mass, less than 1 cm, and gray or yellowish color, and deformed appendix are characteristic findings of appendix cancer.
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| *On microscopic histopathological analysis findings will depend on the subtype of appendicular cancer.
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| *Common histopathological findings, may include:
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| :*Cystic structures
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| :*Angiolymphatic invasion
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| :*Well differentiated and mucinous
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| <gallery>800px-Appendix_Carcinoid_Torsion_1X_PA.JPG | Appendiceal carcinoid<ref name=aaa>http://librepathology.org/wiki/index.php/Neuroendocrine_tumour_of_the_appendix</ref>
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| 800px-Appendix_Carcinoid_HP_14BR---.jpg | Appendiceal carcinoid<ref name=aaa>http://librepathology.org/wiki/index.php/Neuroendocrine_tumour_of_the_appendix</ref>
| | ==[[Appendix cancer classification|Classification]]== |
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| 800px-Appendix_Carcinoid_Necrosis_PA.JPG | Appendiceal carcinoid with necrosis<ref name =aaa>http://librepathology.org/wiki/index.php/Neuroendocrine_tumour_of_the_appendix</ref>
| | ==[[Appendix cancer pathophysiology|Pathophysiology]]== |
| 800px-Appendix_Carcinoid_Synaptophysin_14BR---.jpg | Carcinoid synaptophysin<ref name=aaa>http://librepathology.org/wiki/index.php/Neuroendocrine_tumour_of_the_appendix</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>
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| </gallery>
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| ==Causes== | | ==[[Appendix cancer causes|Causes]]== |
| *Common causes of appendix cancer may include:
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| *Chronic inflammatory diseases, such as:
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| :*Ulcerative colitis
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| :*Crohn's disease
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| *Hereditary syndromes, such as:
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| :*Familial adenomatous polyposis
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| :*Hereditary non-polyposis colorectal cancer
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| ==Differentiating Appendix Cancer from Other Diseases== | | ==[[Appendix cancer differential diagnosis|Differentiating Appendix cancer from other Diseases]]== |
| * Appendix cancer must be differentiated from other diseases that cause abdominal pain, nausea, vomiting such as:
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| :*[[Appendicitis]]
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| :*[[Typhlitis]]
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| :*Infectious terminal ileitis
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| :*Ileocecal enteric duplication cyst
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| ==Epidemiology and Demographics==
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| * The estimated prevalence of appendix cancer is approximately 0.12 cases per 100,000 individuals in the United States.
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| * The estimated prevalence of adenocarcinoma of the appendix is 0.2 cases per 100,000 individuals worldwide.
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| ===Age===
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| * The median age at diagnosis of patients with appendix cancer is 65 years.
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| * Appendix cancer is more commonly observed among patients aged 50 to 60 years old.
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| * Appendix cancer is more commonly observed among adults and elderly patients.
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| ===Gender=== | | ==[[Appendix cancer epidemiology and demographics|Epidemiology and Demographics]]== |
| *Males are more commonly affected with appendix cancer than females.
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| ===Race=== | | ==[[Appendix cancer risk factors|Risk Factors]]== |
| *There is no racial predilection for appendix cancer.
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| ==Risk Factors== | | ==[[Appendix cancer screening|Screening]]== |
| *Common risk factors in the development of appendix cancer are family history of cancer, long-standing ulcerative colitis, and chronic inflammatory bowel diseases.
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| == Natural History, Complications and Prognosis== | | ==[[Appendix cancer natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| *The majority of patients with appendix cancer may be initially asymptomatic.
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| *Early clinical features include periodical unspecific abdominal pain, bloating, and nausea.
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| *If left untreated, the majority of patients with appendix cancer may progress to develop peritoneal carcinomatosis and metastases.
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| *Common complications of appendix cancer include perforation, peritonitis, and abscess.
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| *Prognosis is generally good, and the 5-survival rate of patients with appendix cancer is approximately 90%.
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| == Diagnosis == | | ==Diagnosis== |
| ===Diagnostic Criteria===
| | [[Appendix cancer diagnostic study of choice|Diagnostic study of choice]] | [[Appendix cancer history and symptoms|History and Symptoms]] | [[Appendix cancer physical examination|Physical Examination]] | [[Appendix cancer laboratory findings|Laboratory Findings]] | [[Appendix cancer electrocardiogram|Electrocardiogram]] | [[Appendix cancer x ray|X-Ray Findings]] | [[Appendix cancer echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Appendix cancer CT scan|CT-Scan Findings]] | [[Appendix cancer MRI|MRI Findings]] | [[Appendix cancer other imaging findings|Other Imaging Findings]] | [[Appendix cancer other diagnostic studies|Other Diagnostic Studies]] |
| *The diagnosis of appendix cancer is made with histopathological evaluation of the following diagnostic criteria:
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| :*Tumor confined to the appendix
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| :*Invasion to the muscularis mucosae
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| :*Presence of metastases
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| :*Spread to the peritoneal cavity
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| === Symptoms === | | ==Treatment== |
| *Symptoms of appendix cancer may include the following:
| | [[Appendix cancer medical therapy|Medical Therapy]] | [[Appendix cancer interventions|Interventions]] | [[Appendix cancer surgery|Surgery]] | [[Appendix cancer primary prevention|Primary Prevention]] | [[Appendix cancer secondary prevention|Secondary Prevention]] | [[Appendix cancer cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Appendix cancer future or investigational therapies|Future or Investigational Therapies]] |
| :*Chronic [[abdominal pain]]
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| ::*Periumbilical
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| ::*Right lower quadrant
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| :*[[Loss of appetite]]
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| :*[[Nausea]]
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| :*[[Vomiting]]
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| :*[[Diarrhea]]
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| :*[[Micturition]]
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| :*[[Tenesmus]]
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| :*[[Indigestion]]
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| :*[[Flatulence]]
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| :*Bowel irregularity
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| === Physical Examination === | | ==Case Studies== |
| *Patients with appendix cancer may appear normal, pale or diaphoretic.
| | [[Appendix cancer case study one|Case #1]] |
| *Abdominal examination may be remarkable for:
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| '''Palpation'''
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| *The abdominal wall is very sensitive to mild palpation
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| *[[Rebound tenderness]]
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| *[[Abdominal guarding]]
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| * [[Rovsing's sign]]
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| * [[Psoas sign]]
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| '''Rectal examination''''
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| *Tenderness in the [[rectovesical pouch]]
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| === Laboratory Findings ===
| | [[Category:Surgery]] |
| *Laboratory findings consistent with the diagnosis of appendix cancer, may include:
| | [[Category:Medicine]] |
| :* [[Leukocytosis]] and a shift to the left in the segmented [[neutrophils]].
| | [[Category:Emergency medicine]] |
| :* Elevated serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA) | | [[Category:Oncology]] |
| :* Elevated c-reactive protein (CRP)
| | [[Category:Up-To-Date]] |
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| ===Imaging Findings===
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| *Enhanced CT is the imaging modality of choice for appendix cancer.
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| *On CT, appendix cancer is characterized by the following findings:
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| :*Soft tissue thickening
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| :*Wall irregularity
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| :*Presence of pseudomyxoma peritonei
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| :*Calcification
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| :*Internal septations
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| :*Periappendiceal fat stranding and intraperitoneal free fluid is nonspecific.
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| === Other Diagnostic Studies ===
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| * Appendix cancer may also be diagnosed using the following diagnostic studies:
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| :*[[Somatostatin]] [[scintigraphy]] with 111-indium-octreotide
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| :*[[Bone]] [[scintigraphy]] with 99mTc-methylene diphosphonate (99mTcMDP)
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| :*[[Capsule endoscopy]] | |
| :*[[Enteroscopy]]
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| :*[[Positron emission tomography]] (PET) | |
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| == Treatment ==
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| === Medical Therapy ===
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| *There is no treatment for appendix cancer; the mainstay of therapy is supportive care.
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| *The mainstay of therapy for appendix cancer is [medical therapy 1] and [medical therapy 2].
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| *[Medical therapy 1] acts by [mechanism of action1].
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| *Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
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| === Surgery ===
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| *Surgery is the mainstay of therapy for appendix cancer.
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| *[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of appendix cancer.
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| *[Surgical procedure] can only be performed for patients with [disease stage] appendix cancer.
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| === Prevention ===
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| *There are no primary preventive measures available for appendix cancer.
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| *Effective measures for the primary prevention of appendix cancer include [measure1], [measure2], and [measure3].
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| *Once diagnosed and successfully treated, patients with appendix cancer are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
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| ==References==
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| {{Reflist|2}}
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| [[Category: Oncology]]
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