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| __NOTOC__ | | __NOTOC__ |
| | | {{Small intestine cancer}} |
| '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' | | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{CMG}}; {{AE}} {{Qurrat}}, {{PSD}} | | {{CMG}}; {{AE}} {{Qurrat}}, {{PSD}} |
| {{Small intestine cancer}}
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| {{SK}}
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| ==Overview==
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| ==Historical Perspective==
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| Despite of
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| ==Classification==
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| Intestinal cancers can be classified into:<ref name="pmid11588539">{{cite journal |vauthors=Gill SS, Heuman DM, Mihas AA |title=Small intestinal neoplasms |journal=J. Clin. Gastroenterol. |volume=33 |issue=4 |pages=267–82 |date=October 2001 |pmid=11588539 |doi= |url=}}</ref>
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| *Benign tumors
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| *Malignant tumors
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| *Extra-intestinal tumors.
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| {| class="wikitable"
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| !Tumors
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| !Types
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| |'''Benign Tumors'''
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| |1- Leiomyoma
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| 2-Lipoma
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| 3-Hamartoma
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| 4-Desmoid tumors
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| |'''Malignant Tumors'''
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| |1-Adenocarcinoma
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| 2-Leiomyosarcoma
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| 3-Carcinoid tumors
| | {{SK}} Small bowel cancer |
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| 4-Neuroendocrine tumors
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| 5-Lymphomas
| | ==[[Small intestine cancer overview|Overview]]== |
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| |'''Extra- Intestinal Tumors'''
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| |Metastasis to small intestine is mostly through: | |
| 1-Contagious spread (uncommon, mostly route of spread for Melanoma)
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| 2-Peritoneal spread
| | ==[[Small intestine cancer historical perspective|Historical Perspective]]== |
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| ==Pathophysiology== | | ==[[Small intestine cancer classification|Classification]]== |
| *Pathophysiology of the small intestinal cancers is not much studied domain, as it is a rare condition.
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| *Studies are being conducted to evaluate association with environmental risk factors. <ref name="pmid8850266">{{cite journal |vauthors=Severson RK, Schenk M, Gurney JG, Weiss LK, Demers RY |title=Increasing incidence of adenocarcinomas and carcinoid tumors of the small intestine in adults |journal=Cancer Epidemiol. Biomarkers Prev. |volume=5 |issue=2 |pages=81–4 |date=February 1996 |pmid=8850266 |doi= |url=}}</ref>
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| =====Associations:=====
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| *Cancer of small intestine can arise sporadically or they are associated with genetic diseases.
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| *The pathophysiology of small intestinal cancers depend on the histological subtype.
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| *Duodenal tumors are more common than the tumors of jejunum and illeum.<ref name="pmid15274064">{{cite journal |vauthors=Dabaja BS, Suki D, Pro B, Bonnen M, Ajani J |title=Adenocarcinoma of the small bowel: presentation, prognostic factors, and outcome of 217 patients |journal=Cancer |volume=101 |issue=3 |pages=518–26 |date=August 2004 |pmid=15274064 |doi=10.1002/cncr.20404 |url=}}</ref> Adenomas arise more proximally, in the duodenum and lymphomas arise in jejunum and ileum.<ref name="pmid8921448">{{cite journal |vauthors=Chow JS, Chen CC, Ahsan H, Neugut AI |title=A population-based study of the incidence of malignant small bowel tumours: SEER, 1973-1990 |journal=Int J Epidemiol |volume=25 |issue=4 |pages=722–8 |date=August 1996 |pmid=8921448 |doi= |url=}}</ref> Some of the associations of intestinal cancers are:<ref name="pmid11588539">{{cite journal |vauthors=Gill SS, Heuman DM, Mihas AA |title=Small intestinal neoplasms |journal=J. Clin. Gastroenterol. |volume=33 |issue=4 |pages=267–82 |date=October 2001 |pmid=11588539 |doi= |url=}}</ref>
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| **[[ Familial Adenomatous Polyposis Coli]]
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| ** [[Peutz-Jeghers syndrome]]
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| **Chronic intestinal inflammatory disorders e.g., [[Crohn's disease]] or [[Celiac sprue]]
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| ===Adenocarcinoma:=== | | ==[[Small intestine cancer pathophysiology|Pathophysiology]]== |
| *[[Adenocarcinoma]] of the smalls intestine originate locally or can be associated with malignant tumors of other sites.
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| *Rarely people with [[Peutz-Jeghers syndrome]] can develop malignant changes in polyps present in the small intestine.
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| *Primary [[adenocarcinoma]] consists of 40% of cases of malignant tumors of small intestine and it is the most common histologic type. <ref name="pmid6821853">{{cite journal |vauthors=Barclay TH, Schapira DV |title=Malignant tumors of the small intestine |journal=Cancer |volume=51 |issue=5 |pages=878–81 |date=March 1983 |pmid=6821853 |doi= |url=}}</ref>
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| ===Neuroendocrine tumors:=== | | ==[[Small intestine cancer causes|Causes]]== |
| *Small Intestinal neuroendocrine tumors (SI-NETs) are the most common gastrointestinal neuroendocrine tumors.<ref name="ModlinChampaneria2007">{{cite journal|last1=Modlin|first1=Irvin M.|last2=Champaneria|first2=Manish C.|last3=Chan|first3=Anthony K.C.|last4=Kidd|first4=Mark|title=A Three-Decade Analysis of 3,911 Small Intestinal Neuroendocrine Tumors: The Rapid Pace of No Progress|journal=The American Journal of Gastroenterology|volume=102|issue=7|year=2007|pages=1464–1473|issn=0002-9270|doi=10.1111/j.1572-0241.2007.01185.x}}</ref>
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| *They are diverse neoplasms emerging from the endocrine cells of the intestinal mucosa.<ref name="pmid28210937">{{cite journal |vauthors=Massironi S, Del Gobbo A, Cavalcoli F, Fiori S, Conte D, Pellegrinelli A, Milione M, Ferrero S |title=IMP3 expression in small-intestine neuroendocrine neoplasms: a new predictor of recurrence |journal=Endocrine |volume=58 |issue=2 |pages=360–367 |date=November 2017 |pmid=28210937 |doi=10.1007/s12020-017-1249-x |url=}}</ref>
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| *These tumors originate from enterochromaffin (EC) cells and secrete serotonin.<ref name="pmid27003604">{{cite journal |vauthors=Sei Y, Feng J, Zhao X, Forbes J, Tang D, Nagashima K, Hanson J, Quezado MM, Hughes MS, Wank SA |title=Polyclonal Crypt Genesis and Development of Familial Small Intestinal Neuroendocrine Tumors |journal=Gastroenterology |volume=151 |issue=1 |pages=140–51 |date=July 2016 |pmid=27003604 |pmc=5578471 |doi=10.1053/j.gastro.2016.03.007 |url=}}</ref>
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| *The oncofetal protein IMP3 is a marker that plays a role in the growth of neuroendocrine cells and its expression is associated with prognostic value in neuroendocrine tumors.<ref name="pmid28210937">{{cite journal |vauthors=Massironi S, Del Gobbo A, Cavalcoli F, Fiori S, Conte D, Pellegrinelli A, Milione M, Ferrero S |title=IMP3 expression in small-intestine neuroendocrine neoplasms: a new predictor of recurrence |journal=Endocrine |volume=58 |issue=2 |pages=360–367 |date=November 2017 |pmid=28210937 |doi=10.1007/s12020-017-1249-x |url=}}</ref>
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| [[File:Macroscopic-appearance-of-neuroendocrine-tumor-recurrence.jpg|300px|thumb|none|Neuroendocrine tumor of the small intestine, Macroscopic appearance[https://www.researchgate.net/figure/Macroscopic-appearance-of-neuroendocrine-tumor-recurrence_fig5_268752573 <ref name="pmid25437652">{{Cite pmid|25437652}}</ref>]]] | | ==[[Small intestine cancer differential diagnosis|Differentiating Small Intestine Cancer from other Diseases]]== |
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| ====Carcinoid tumors:==== | | ==[[Small intestine cancer epidemiology and demographics|Epidemiology and Demographics]]== |
| *[[Carcinoid tumors]] are the second most common cancer of the small intestine.
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| *It is a slow growing tumor of small intestine arising as a subset of neuroendocrine cells.
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| *[[Carcinoid tumors]] of the smalls intestine are mostly associated with malignant tumors of the other sites. <ref name="pmid6821853">{{cite journal |vauthors=Barclay TH, Schapira DV |title=Malignant tumors of the small intestine |journal=Cancer |volume=51 |issue=5 |pages=878–81 |date=March 1983 |pmid=6821853 |doi= |url=}}</ref>
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| [[File:Carcinoid Tumor of the Small Intestine.jpeg|300px|thumb|none|Carcinoid tumor of small intestine invading plica circularis[https://commons.wikimedia.org/wiki/File:Multiple_Carcinoid_Tumors_of_the_Small_Bowel_2.jpg Source: Wikimedia commons]]] | | ==[[Small intestine cancer risk factors|Risk Factors]]== |
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| ===Non-Hodgkin Lymphoma:=== | | ==[[Small intestine cancer screening|Screening]]== |
| *After stomach, small intestine is the most common extra-nodal site of presentation of non-Hodgkin lymphomas and it represents 4% to 20% of all the non-Hodgkin lymphomas.
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| *Some of the association of non-Hodgkin lymphomas are :<ref name="pmid10375089">{{cite journal |vauthors=Crump M, Gospodarowicz M, Shepherd FA |title=Lymphoma of the gastrointestinal tract |journal=Semin. Oncol. |volume=26 |issue=3 |pages=324–37 |date=June 1999 |pmid=10375089 |doi= |url=}}</ref>
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| **Helicobacter pylori infection
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| **Immunosuppression after solid-organ transplantation
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| **Celiac disease
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| **Inflammatory bowel disease
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| **Human immunodeficiency virus (HIV)
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| *Small intestinal lymphomas are of low-grade histology and arise from mucosal-associated lymphoid tissues (MALT) present in ileum and jejunum.Sometimies distinct clinicopathologic entities arise from these mucosal-associated lymphoid tissues (MALT), such as immunoproliferative small intestinal disease, primary intestinal T-cell lymphoma and multiple lymphomatous polyposis.<ref name="pmid10375089">{{cite journal |vauthors=Crump M, Gospodarowicz M, Shepherd FA |title=Lymphoma of the gastrointestinal tract |journal=Semin. Oncol. |volume=26 |issue=3 |pages=324–37 |date=June 1999 |pmid=10375089 |doi= |url=}}</ref>
| | ==[[Small intestine cancer natural history, complications, and prognosis|Natural History, Complications, and Prognosis]]== |
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| ===Small intestinal stromal tumors (GISTs):===
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| *Gastrointestinal stromal tumors (GISTs) are the most common benign tumors of the gastrointestinal (GI) tract.
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| *GISTs typically develop in older age. Stromal tumors are most common in the stomach, 60–70% of the stromal tumors, followed by small intestine which makes 20–25% of all the stromal tumors of GI tract, colon and rectum makes 5% and esophagus is less than 5%. GISTs are mostly benign tumors and rarely can be malignant.
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| *Instestinal stromal cell tumors should be differentiated from leiomyosarcomas and leiomyomas. They differ clinically and pathogenetically from leiomyosarcomas and leiomyomas. Leimyomas occur in the GI tract, commonly in the esophagus.
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| *One of the subset of intestinal stromal cell tumors is the GI autonomic nerve tumors (GANTs). Stromal tumors can be differentiated from other tumors of small intestine by their cell specific markers.
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| *GISTs express following stromal cell markers:<ref name="pmid1432-2307">{{cite journal| author=Schlotzhauer WS, Chortyk OT, Austin PR| title=Pyrolysis of chitin, a potential tobacco extender. | journal=J Agric Food Chem | year= 1976 | volume= 24 | issue= 1 | pages= 177-80 | pmid=1432-2307 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1432 }} </ref>
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| **70% of GISTs are positive for CD34
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| **20–30% are positive for smooth muscle actin (SMA)
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| **10% are positive for S100 protein
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| **<5% are positive for desmin
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| ==Causes==
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| *The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].
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| *The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click [[Pericarditis causes#Overview|here]].
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| ==Epidemiology and Demographics==
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| *Small intestinal cancer makes less than two percent of the gastrointestinal track cancers.<ref name="pmid10651347">{{cite journal |vauthors=North JH, Pack MS |title=Malignant tumors of the small intestine: a review of 144 cases |journal=Am Surg |volume=66 |issue=1 |pages=46–51 |date=January 2000 |pmid=10651347 |doi= |url=}}</ref>
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| *There incidence has increased recently particularly in African American males and it is unchanged for the females.<ref name="pmid8850266">{{cite journal |vauthors=Severson RK, Schenk M, Gurney JG, Weiss LK, Demers RY |title=Increasing incidence of adenocarcinomas and carcinoid tumors of the small intestine in adults |journal=Cancer Epidemiol. Biomarkers Prev. |volume=5 |issue=2 |pages=81–4 |date=February 1996 |pmid=8850266 |doi= |url=}}</ref>
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| ==Risk Factors==
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| *There are no established risk factors for small intestinal cancers.
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| *[[Crohn disease]] is considered to be the most important risk factor of small intestinal cancers.<ref name="pmid15274064">{{cite journal |vauthors=Dabaja BS, Suki D, Pro B, Bonnen M, Ajani J |title=Adenocarcinoma of the small bowel: presentation, prognostic factors, and outcome of 217 patients |journal=Cancer |volume=101 |issue=3 |pages=518–26 |date=August 2004 |pmid=15274064 |doi=10.1002/cncr.20404 |url=}}</ref>
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| *Other associated risk factors are: <ref name="pmid29498365">{{cite journal |vauthors=Sarosiek T, Stelmaszuk M |title=[Small intestine neoplasms] |language=Polish |journal=Pol. Merkur. Lekarski |volume=44 |issue=260 |pages=45–48 |date=February 2018 |pmid=29498365 |doi= |url=}}</ref>
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| **[[Familial Adenomatous Polyposis]] (FAP)
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| **[[Lynch syndrome]]
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| **[[Acquired immunodeficiency syndrome]] (AIDS)
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| **[[Celiac disease]]
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| **[[Peutz-Jeghers Syndrome]]
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| **[[Hereditary nonpolyposis colorectal neoplasms]]
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| ==Screening==
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| *Currently there are no screening protocols and rarity of condition makes it a less suspected condition.<ref name="pmid9834319">{{cite journal |vauthors=Rossini FP, Risio M, Pennazio M |title=Small bowel tumors and polyposis syndromes |journal=Gastrointest. Endosc. Clin. N. Am. |volume=9 |issue=1 |pages=93–114 |date=January 1999 |pmid=9834319 |doi= |url=}}</ref>
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| ==Natural History, Complications, and Prognosis==
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| *Clinical features and natural history of small intestinal tumors have not been clearly studied, as its a rare condition.<ref name="pmid20871040">{{cite journal |vauthors=Higashi D, Ishibashi Y, Tamura T, Nii K, Egawa Y, Koga M, Tomiyasu T, Harimura T, Tanaka R, Futatsuki R, Noda S, Futami K, Maekawa T, Takaki Y, Hirai F, Matsui T |title=Clinical features of and chemotherapy for cancer of the small intestine |journal=Anticancer Res. |volume=30 |issue=8 |pages=3193–7 |date=August 2010 |pmid=20871040 |doi= |url=}}</ref>
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| *Adenocarcinoma of the duodenum is associated with low overall survival rate compared to the tumors located in Jejunum and ileum.<ref name="pmid15274064">{{cite journal |vauthors=Dabaja BS, Suki D, Pro B, Bonnen M, Ajani J |title=Adenocarcinoma of the small bowel: presentation, prognostic factors, and outcome of 217 patients |journal=Cancer |volume=101 |issue=3 |pages=518–26 |date=August 2004 |pmid=15274064 |doi=10.1002/cncr.20404 |url=}}</ref>
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| ==Diagnosis== | | ==Diagnosis== |
| *Adenocarcinoma of the small intestine is usually diagnosed late and patients present with metastasis of lymph node or distant sites.
| | [[Small intestine cancer diagnostic study of choice|Diagnostic study of choice]] | [[Small intestine cancer history and symptoms|History and Symptoms]] | [[Small intestine cancer physical examination|Physical Examination]] | [[Small intestine cancer laboratory findings|Laboratory Findings]] | [[Small intestine cancer electrocardiogram|Electrocardiogram]] | [[Small intestine cancer x ray|X-Ray Findings]] | [[Small intestine cancer echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Small intestine cancer CT|CT-Scan Findings]] | [[Small intestine cancer MRI|MRI Findings]] | [[Small intestine cancer other imaging findings|Other Imaging Findings]] | [[Small intestine cancer other diagnostic studies|Other Diagnostic Studies]] |
| *Main stay of the treatment is surgery for these tumors. <ref name="pmid20716869">{{cite journal |vauthors=Ogata Y, Yamaguchi K, Sasatomi T, Uchida S, Akagi Y, Shirouzu K |title=[Treatment and outcome in small bowel cancer] |language=Japanese |journal=Gan To Kagaku Ryoho |volume=37 |issue=8 |pages=1454–7 |date=August 2010 |pmid=20716869 |doi= |url=}}</ref>.
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| *Small intestinal cancers are not suspected clinically as their incidence is very low.
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| *Inaccessibility of endoscope to the small intestine can be the cause of their late diagnosis.
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| *Small intestinal tumors can be diagnosed using enteroscope.<ref name="pmid9834319">{{cite journal |vauthors=Rossini FP, Risio M, Pennazio M |title=Small bowel tumors and polyposis syndromes |journal=Gastrointest. Endosc. Clin. N. Am. |volume=9 |issue=1 |pages=93–114 |date=January 1999 |pmid=9834319 |doi= |url=}}</ref>
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| ===History and Symptoms===
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| *The majority of patients with small Intestinal cancers are asymptomatic or have non-specific symptoms, which contributes to its late diagnosis and more investigations.<ref name="pmid24701741">{{cite journal |vauthors=Williamson JM, Williamson RC |title=Small bowel tumors: pathology and management |journal=J Med Assoc Thai |volume=97 |issue=1 |pages=126–37 |date=January 2014 |pmid=24701741 |doi= |url=}}</ref>
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| ===Physical Examination===
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| *Clinical features of small intestine tumors have not been clearly studied, as its a rare condition.<ref name="pmid20871040">{{cite journal |vauthors=Higashi D, Ishibashi Y, Tamura T, Nii K, Egawa Y, Koga M, Tomiyasu T, Harimura T, Tanaka R, Futatsuki R, Noda S, Futami K, Maekawa T, Takaki Y, Hirai F, Matsui T |title=Clinical features of and chemotherapy for cancer of the small intestine |journal=Anticancer Res. |volume=30 |issue=8 |pages=3193–7 |date=August 2010 |pmid=20871040 |doi= |url=}}</ref>
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| *
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| ===Ultrasonography===
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| *Ultrasonography can provide useful information in the diagnosis as small intestinal tumors have typical ultrasonographic features.
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| *
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| ===CT scan===
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| *CT can help in differenting the small intestinal cancers. Different tumors have different appearance on CT:<ref name="BuckleyFishman1998">{{cite journal|last1=Buckley|first1=J A|last2=Fishman|first2=E K|title=CT evaluation of small bowel neoplasms: spectrum of disease.|journal=RadioGraphics|volume=18|issue=2|year=1998|pages=379–392|issn=0271-5333|doi=10.1148/radiographics.18.2.9536485}}</ref>
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| {| class="wikitable"
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| !Tumors
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| !Findings on CT
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| |'''Adenocarcinmoa'''
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| |Ulcerative lesions, nodular lesions and annular lesions
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| |'''Carcinoid tumors'''
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| |Poorly defined homogenous mass displacing intestinal loops
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| Calcification and desmoplastic reaction is a very specific finding of [[Carcinoid tumors]] on CT
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| |'''Non-Hodgkin lymphoma '''
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| |Bulky mass on CT
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| Lymphoma is associated with significant dilation of the lumen of intestine
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| |'''Gastrointestinal stromal tumors(GISTs) '''
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| |Intraluminal, subserosal or submucosal homogeneous masses with sharply defined borders
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| Calcifications
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| |'''Lipoma '''
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| |Intraluminal homogeneous mass, well-circumscribed and with some fat attenuation
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| |'''Malignant metastasized tumors '''
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| |Intraperitoneal seedings extending locally
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| Metastasized tumors give appearance of multiple small nodular masses in intestinal serosa, mesentery and omentum.
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| |} | |
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| *In [[Peutz-Jeghers Syndrome]], non-neoplastic lesions may resemble small intestinal neoplasm on CT.
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| ===MRI===
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| ===Enteroscopy===
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| ===Biopsy===
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| ==Treatment== | | ==Treatment== |
| | [[Small intestine cancer medical therapy|Medical Therapy]] | [[Small intestine cancer surgery|Surgery]] | [[Small intestine cancer primary prevention|Primary Prevention]] | [[Small intestine cancer secondary prevention|Secondary Prevention]] | [[Small intestine cancer cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Small intestine cancer future or investigational therapies|Future or Investigational Therapies]] |
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| ===Medical Treatment=== | | ==Case Studies== |
| | | [[Small intestine cancer case study one|Case #1]] |
| ===Surgical Treatment===
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| *Primary treatment for cancer of the small intestine is curative surgical resection.<ref name="pmid20871040">{{cite journal |vauthors=Higashi D, Ishibashi Y, Tamura T, Nii K, Egawa Y, Koga M, Tomiyasu T, Harimura T, Tanaka R, Futatsuki R, Noda S, Futami K, Maekawa T, Takaki Y, Hirai F, Matsui T |title=Clinical features of and chemotherapy for cancer of the small intestine |journal=Anticancer Res. |volume=30 |issue=8 |pages=3193–7 |date=August 2010 |pmid=20871040 |doi= |url=}}</ref>
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| *For non-metastatic disease curative surgery gives good results, when done in a tertiary care center.<ref name="pmid15274064">{{cite journal |vauthors=Dabaja BS, Suki D, Pro B, Bonnen M, Ajani J |title=Adenocarcinoma of the small bowel: presentation, prognostic factors, and outcome of 217 patients |journal=Cancer |volume=101 |issue=3 |pages=518–26 |date=August 2004 |pmid=15274064 |doi=10.1002/cncr.20404 |url=}}</ref>
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| *Adenocarcinoma of duodenum has a comparitive low 5-year disease free survival rate after surgery.<ref name="pmid15274064">{{cite journal |vauthors=Dabaja BS, Suki D, Pro B, Bonnen M, Ajani J |title=Adenocarcinoma of the small bowel: presentation, prognostic factors, and outcome of 217 patients |journal=Cancer |volume=101 |issue=3 |pages=518–26 |date=August 2004 |pmid=15274064 |doi=10.1002/cncr.20404 |url=}}</ref>
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| ===Radiotherapy===
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| ===Chemotherapy===
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| *The role of adjuvant chemotherapy for small intestinal cancer is not established yet.<ref name="pmid20397775">{{cite journal |vauthors=Overman MJ, Kopetz S, Lin E, Abbruzzese JL, Wolff RA |title=Is there a role for adjuvant therapy in resected adenocarcinoma of the small intestine |journal=Acta Oncol |volume=49 |issue=4 |pages=474–9 |date=May 2010 |pmid=20397775 |doi=10.3109/02841860903490051 |url=}}</ref>
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| *when resection is not possible, palliative surgery and chemotherapy are other options.<ref name="pmid20871040">{{cite journal |vauthors=Higashi D, Ishibashi Y, Tamura T, Nii K, Egawa Y, Koga M, Tomiyasu T, Harimura T, Tanaka R, Futatsuki R, Noda S, Futami K, Maekawa T, Takaki Y, Hirai F, Matsui T |title=Clinical features of and chemotherapy for cancer of the small intestine |journal=Anticancer Res. |volume=30 |issue=8 |pages=3193–7 |date=August 2010 |pmid=20871040 |doi= |url=}}</ref>
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| ===Prevention===
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| ===Primary Prevention===
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| *There are no established measures for the primary prevention of small intestinal cancers.
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| ===Secondary Prevention===
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| *There are no established measures for the secondary prevention of small intestinal cancers.
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| ==References==
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| {{reflist|2}}
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