|
|
| (40 intermediate revisions by 4 users not shown) |
| Line 1: |
Line 1: |
| __NOTOC__ | | __NOTOC__ |
| {{SI}} | | {{Appendix cancer}} |
| {{CMG}} {{AE}} {{MV}}
| | '''For patient information, click [[Appendix cancer (patient information)|here]]''' |
|
| |
| {{SK}} 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.<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.
| |
|
| |
|
| ==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>
| |
|
| |
|
| ==Classification== | | ==[[Appendix cancer overview|Overview]]== |
| *Appendix cancer may be classified according to WHO classification into 4 groups:
| |
| :*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
| |
| {|style="border: 5px; font-size: 90%; margin: 5px; width: 800px" 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>'''}}
| |
| |valign=top|
| |
| |+
| |
| |-
| |
| | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;"| 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
| |
| |-
| |
| | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;" | Non-epithelial tumors
| |
| |-
| |
| |
| |
| *Neuroma
| |
| *Lipoma
| |
| *Leiomyoma
| |
| *Gastrointestinal stromal tumor
| |
| *Leiomyosarcoma
| |
| *Kaposi sarcoma
| |
| *Others
| |
| |-
| |
| |style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;"| Secondary tumors
| |
| |-
| |
| |
| |
| *Metastasis (eg. Primary of urogenital tract, breast, lung)
| |
| |-
| |
| |style="padding: 5px 5px; background: #DCDCDC; font-weight: bold; text-align:center;"| Hyperplastic polyp
| |
| |}
| |
|
| |
|
| ==Pathophysiology== | | ==[[Appendix cancer historical perspective|Historical Perspective]]== |
| *The pathogenesis of appendix cancer is characterized by an initial epithelial dysplasia.
| |
| *The KRAS gene mutation has been associated with the development of appendix cancer.
| |
| *On gross pathology, well-demarcated mass, less than 1 cm, and gray or yellowish color, and deformed appendix are characteristic findings of appendix cancer.
| |
| *On microscopic histopathological analysis findings will depend on the subtype of appendicular cancer.
| |
| *Common histopathological findings, may include:
| |
| :*Cystic structures
| |
| :*Angiolymphatic invasion
| |
| :*Well differentiated and mucinous
| |
| <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>
| |
|
| |
|
| 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]]== |
|
| |
|
| 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>
| |
| 800px-Appendix_Carcinoid_HP_CTR.jpg | Appendiceal tumor<ref name=aaa>http://librepathology.org/wiki/index.php/Neuroendocrine_tumour_of_the_appendix</ref>
| |
| </gallery>
| |
|
| |
|
| ==Causes== | | ==[[Appendix cancer causes|Causes]]== |
| *Common causes of appendix cancer may include:
| |
| *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 differential diagnosis|Differentiating Appendix cancer from other Diseases]]== |
| * Appendix cancer must be differentiated from other diseases that cause abdominal pain, nausea, vomiting such as:
| |
| :*[[Appendicitis]]
| |
| :*[[Typhlitis]]
| |
| :*Infectious terminal ileitis
| |
| :*Ileocecal enteric duplication cyst
| |
|
| |
| ==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 adenocarcinoma of the appendix is 0.2 cases per 100,000 individuals worldwide.
| |
| ===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=== | | ==[[Appendix cancer epidemiology and demographics|Epidemiology and Demographics]]== |
| *Males are more commonly affected with appendix cancer than females.
| |
|
| |
|
| ===Race=== | | ==[[Appendix cancer risk factors|Risk Factors]]== |
| *There is no racial predilection for appendix cancer.
| |
|
| |
|
| ==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.
| |
|
| |
|
| == 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.
| |
| *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.
| |
| *Common complications of appendix cancer include perforation, peritonitis, and abscess.
| |
| *Prognosis is generally good, and the 5-survival rate of patients with appendix cancer is approximately 90%.
| |
|
| |
|
| == 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:
| |
| :*Tumor confined to the appendix
| |
| :*Invasion to the muscularis mucosae
| |
| :*Presence of metastases
| |
| :*Spread to the peritoneal cavity
| |
|
| |
|
| === 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]]
| |
| ::*Periumbilical
| |
| ::*Right lower quadrant
| |
| :*[[Loss of appetite]]
| |
| :*[[Nausea]]
| |
| :*[[Vomiting]]
| |
| :*[[Diarrhea]]
| |
| :*[[Micturition]]
| |
| :*[[Tenesmus]]
| |
| :*[[Indigestion]]
| |
| :*[[Flatulence]]
| |
| :*Bowel irregularity
| |
|
| |
|
| === 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:
| |
| '''Palpation'''
| |
| *The abdominal wall is very sensitive to mild palpation
| |
| *[[Rebound tenderness]]
| |
| *[[Abdominal guarding]]
| |
| * [[Rovsing's sign]]
| |
| * [[Psoas sign]]
| |
| '''Rectal examination''''
| |
| *Tenderness in the [[rectovesical pouch]]
| |
|
| |
|
| === 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]] |
| | |
| ===Imaging Findings===
| |
| *Enhanced CT is the imaging modality of choice for appendix cancer.
| |
| *On CT, appendix cancer is characterized by the following findings:<ref name="pmid16973770">{{cite journal |vauthors=Hoeffel C, Crema MD, Belkacem A, Azizi L, Lewin M, Arrivé L, Tubiana JM |title=Multi-detector row CT: spectrum of diseases involving the ileocecal area |journal=Radiographics |volume=26 |issue=5 |pages=1373–90 |year=2006 |pmid=16973770 |doi=10.1148/rg.265045191 |url=}}</ref>
| |
| :*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:
| |
| :*[[Somatostatin]] [[scintigraphy]] with 111-indium-octreotide
| |
| :*[[Bone]] [[scintigraphy]] with 99mTc-methylene diphosphonate (99mTcMDP) | |
| :*[[Capsule endoscopy]]
| |
| :*[[Enteroscopy]] | |
| :*[[Positron emission tomography]] (PET)
| |
| | |
| == Treatment ==
| |
| === Medical Therapy ===
| |
| *The mainstay medical therapy for appendix cancer is supportive care.
| |
| *Supportive care for appendix cancer, may include:
| |
| | |
| === 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 12 months.
| |
| *Follow-up testing includes physical examination, abdominal ultrasound, and laboratory studies (eg. tumoral biomarkers)
| |
| | |
| ==References==
| |
| {{Reflist|2}}
| |
|
| |
| [[Category: Oncology]]
| |