Linitis plastica pathophysiology: Difference between revisions
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==Pathogenesis== | ==Pathogenesis== | ||
*Linitis plastica is a morphological variant of diffuse (or infiltrating) gastric adenocarcinoma.<ref name=lp>Linitis plastica. Wikipedia. https://en.wikipedia.org/wiki/Linitis_plastica Accessed on November 12, 2015.</ref> | *Linitis plastica is a morphological variant of diffuse (or infiltrating) gastric adenocarcinoma.<ref name=lp>Linitis plastica. Wikipedia. https://en.wikipedia.org/wiki/Linitis_plastica Accessed on November 12, 2015.</ref> | ||
* Linitis plastica may also occur secondary to metastasis from other primary cancers, particularly infiltrating lobular carcinoma of breast. | * Linitis plastica may also occur secondary to metastasis from other primary cancers, particularly infiltrating lobular carcinoma of breast. | ||
==Genetics== | |||
*[[Germline]] mutations in the ''CDH1'' ([[E-cadherin]]) gene, which results in an altered intercellular adhesions, may be involved in the pathogenesis of diffuse gastric adenocarcinoma.<ref name=mk>Gastric linitis plastica. Orphanet. http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=36273 Accessed on November 19, 2015.</ref><ref name="WolfGeigl2010">{{cite journal|last1=Wolf|first1=E.-M.|last2=Geigl|first2=J.B.|last3=Svrcek|first3=M.|last4=Vieth|first4=M.|last5=Langner|first5=C.|title=Hereditäres Magenkarzinom|journal=Der Pathologe|volume=31|issue=6|year=2010|pages=423–429|issn=0172-8113|doi=10.1007/s00292-010-1353-7}}</ref> | *[[Germline]] mutations in the ''CDH1'' ([[E-cadherin]]) gene, which results in an altered intercellular adhesions, may be involved in the pathogenesis of diffuse gastric adenocarcinoma.<ref name=mk>Gastric linitis plastica. Orphanet. http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=36273 Accessed on November 19, 2015.</ref><ref name="WolfGeigl2010">{{cite journal|last1=Wolf|first1=E.-M.|last2=Geigl|first2=J.B.|last3=Svrcek|first3=M.|last4=Vieth|first4=M.|last5=Langner|first5=C.|title=Hereditäres Magenkarzinom|journal=Der Pathologe|volume=31|issue=6|year=2010|pages=423–429|issn=0172-8113|doi=10.1007/s00292-010-1353-7}}</ref> | ||
*Hereditary form of diffuse gastric adenocarcinoma is transmitted in an [[autosomal dominant]] fashion, with a 70% disease [[penetrance]].<ref name=mk>Gastric linitis plastica. Orphanet. http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=36273 Accessed on November 19, 2015.</ref><ref name="WolfGeigl2010">{{cite journal|last1=Wolf|first1=E.-M.|last2=Geigl|first2=J.B.|last3=Svrcek|first3=M.|last4=Vieth|first4=M.|last5=Langner|first5=C.|title=Hereditäres Magenkarzinom|journal=Der Pathologe|volume=31|issue=6|year=2010|pages=423–429|issn=0172-8113|doi=10.1007/s00292-010-1353-7}}</ref> | *Hereditary form of diffuse gastric adenocarcinoma is transmitted in an [[autosomal dominant]] fashion, with a 70% disease [[penetrance]].<ref name=mk>Gastric linitis plastica. Orphanet. http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=36273 Accessed on November 19, 2015.</ref><ref name="WolfGeigl2010">{{cite journal|last1=Wolf|first1=E.-M.|last2=Geigl|first2=J.B.|last3=Svrcek|first3=M.|last4=Vieth|first4=M.|last5=Langner|first5=C.|title=Hereditäres Magenkarzinom|journal=Der Pathologe|volume=31|issue=6|year=2010|pages=423–429|issn=0172-8113|doi=10.1007/s00292-010-1353-7}}</ref> | ||
==Gross Pathology== | |||
*On gross pathology, thick, rigid, leather bottle-like stomach from diffuse infiltration of the stomach wall is a characteristic finding of linitis plastica.<ref name=lp>Linitis plastica. Wikipedia. https://en.wikipedia.org/wiki/Linitis_plastica Accessed on November 18, 2015.</ref> | *On gross pathology, thick, rigid, leather bottle-like stomach from diffuse infiltration of the stomach wall is a characteristic finding of linitis plastica.<ref name=lp>Linitis plastica. Wikipedia. https://en.wikipedia.org/wiki/Linitis_plastica Accessed on November 18, 2015.</ref> | ||
*[[Metastasis]] to the [[peritoneum]], [[lymph node]]s | *[[Metastasis]] to the [[peritoneum]], [[lymph node]]s, and/or other organs usually occurs by the time linitis plastica is diagnosed.<ref name=mk>Gastric linitis plastica. Orphanet. http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=36273 Accessed on December 7, 2015.</ref> | ||
<gallery> | <gallery> | ||
image:Linitis_plastica1.jpg|Endoscopic image of linitis plastica, a type of stomach cancer where the entire stomach is invaded, leading to a leather bottle-like appearance with blood coming out of it.<ref>Linitis Plastica. Wikimedia. https://en.wikipedia.org/wiki/Linitis_plastica#/media/File:Linitis_plastica_2.jpg Accessed on December 13, 2015</ref></gallery> | image:Linitis_plastica1.jpg|Endoscopic image of linitis plastica, a type of stomach cancer where the entire stomach is invaded, leading to a leather bottle-like appearance with blood coming out of it.<ref>Linitis Plastica. Wikimedia. https://en.wikipedia.org/wiki/Linitis_plastica#/media/File:Linitis_plastica_2.jpg Accessed on December 13, 2015</ref></gallery> | ||
==Microscopic Pathology== | |||
*In diffuse type adenocarcinoma (mucinous, [[colloid]]), tumour cells are discohesive and secrete mucus, which is delivered in the [[interstitium]], producing large pools of mucus or colloid (optically "empty" spaces). If the mucus remains inside the tumour cell, it pushes the nucleus to the periphery, leaving a "signet-ring cell" appearance. | *In diffuse type adenocarcinoma (mucinous, [[colloid]]), tumour cells are discohesive and secrete mucus, which is delivered in the [[interstitium]], producing large pools of mucus or colloid (optically "empty" spaces). If the mucus remains inside the tumour cell, it pushes the nucleus to the periphery, leaving a "signet-ring cell" appearance. | ||
*On microscopic histopathological analysis, atypical [[signet ring cell]]s that diffusely infiltrate the stomach wall, [[submucosal]] [[fibrosis]] and thickening, and a minimal [[mucosa]]l involvement are characteristic findings of linitis plastica.<ref name="SchauerPeiper2011">{{cite journal|last1=Schauer|first1=M|last2=Peiper|first2=M|last3=Theisen|first3=J|last4=Knoefel|first4=W|title=Prognostic factors in patients with diffuse type gastric cancer (linitis plastica) after operative treatment|journal=European Journal of Medical Research|volume=16|issue=1|year=2011|pages=29|issn=2047-783X|doi=10.1186/2047-783X-16-1-29}}</ref><ref>Stomach cancer. Wikipedia. https://en.wikipedia.org/wiki/Stomach_cancer Accessed on November 19, 2015.</ref> | *On microscopic histopathological analysis, atypical [[signet ring cell]]s that diffusely infiltrate the stomach wall, [[submucosal]] [[fibrosis]] and thickening, and a minimal [[mucosa]]l involvement are characteristic findings of linitis plastica.<ref name="SchauerPeiper2011">{{cite journal|last1=Schauer|first1=M|last2=Peiper|first2=M|last3=Theisen|first3=J|last4=Knoefel|first4=W|title=Prognostic factors in patients with diffuse type gastric cancer (linitis plastica) after operative treatment|journal=European Journal of Medical Research|volume=16|issue=1|year=2011|pages=29|issn=2047-783X|doi=10.1186/2047-783X-16-1-29}}</ref><ref>Stomach cancer. Wikipedia. https://en.wikipedia.org/wiki/Stomach_cancer Accessed on November 19, 2015.</ref> | ||
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}}</ref> | }}</ref> | ||
==Gallery== | |||
<gallery> | <gallery> | ||
image:Gastric_signet_ring_cell_carcinoma.jpg|A signet ring cell carcinoma of the stomach. Signet ring cells are seen in the lower half of the image. Gastric epithelium is seen in the upper half of the image. H&E stain.<ref>Linitis Plastica. Wikimedia. https://en.wikipedia.org/wiki/Signet_ring_cell_carcinoma#/media/File:Signet_ring_cell_carcinoma_-_very_high_mag.jpg Accessed on December 13, 2015</ref> | image:Gastric_signet_ring_cell_carcinoma.jpg|A signet ring cell carcinoma of the stomach. Signet ring cells are seen in the lower half of the image. Gastric epithelium is seen in the upper half of the image. H&E stain.<ref>Linitis Plastica. Wikimedia. https://en.wikipedia.org/wiki/Signet_ring_cell_carcinoma#/media/File:Signet_ring_cell_carcinoma_-_very_high_mag.jpg Accessed on December 13, 2015</ref> | ||
Revision as of 15:58, 20 January 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]
Overview
On gross pathology, a thick, rigid, and leather bottle-like stomach wall is a characteristic finding of linitis plastica.[1] On microscopic histopathological analysis, atypical signet ring cells that diffusely infiltrate the stomach wall, submucosal fibrosis and thickening, and a minimal mucosal involvement are characteristic findings of linitis plastica. Development of linitis plastica is the result of genetic mutation in the CDH1 (E-cadherin) gene, that is responsible for intercellular adhesions.[2]
Pathogenesis
- Linitis plastica is a morphological variant of diffuse (or infiltrating) gastric adenocarcinoma.[1]
- Linitis plastica may also occur secondary to metastasis from other primary cancers, particularly infiltrating lobular carcinoma of breast.
Genetics
- Germline mutations in the CDH1 (E-cadherin) gene, which results in an altered intercellular adhesions, may be involved in the pathogenesis of diffuse gastric adenocarcinoma.[2][3]
- Hereditary form of diffuse gastric adenocarcinoma is transmitted in an autosomal dominant fashion, with a 70% disease penetrance.[2][3]
Gross Pathology
- On gross pathology, thick, rigid, leather bottle-like stomach from diffuse infiltration of the stomach wall is a characteristic finding of linitis plastica.[1]
- Metastasis to the peritoneum, lymph nodes, and/or other organs usually occurs by the time linitis plastica is diagnosed.[2]
-
![Endoscopic image of linitis plastica, a type of stomach cancer where the entire stomach is invaded, leading to a leather bottle-like appearance with blood coming out of it.[4]](/images/7/76/Linitis_plastica1.jpg)
Endoscopic image of linitis plastica, a type of stomach cancer where the entire stomach is invaded, leading to a leather bottle-like appearance with blood coming out of it.[4]
![Endoscopic image of linitis plastica, a type of stomach cancer where the entire stomach is invaded, leading to a leather bottle-like appearance with blood coming out of it.[4]](/index.php/File:Linitis_plastica1.jpg)
Microscopic Pathology
- In diffuse type adenocarcinoma (mucinous, colloid), tumour cells are discohesive and secrete mucus, which is delivered in the interstitium, producing large pools of mucus or colloid (optically "empty" spaces). If the mucus remains inside the tumour cell, it pushes the nucleus to the periphery, leaving a "signet-ring cell" appearance.
- On microscopic histopathological analysis, atypical signet ring cells that diffusely infiltrate the stomach wall, submucosal fibrosis and thickening, and a minimal mucosal involvement are characteristic findings of linitis plastica.[5][6]
- Superficial mucosal layers are usually spared and rarely show superficial ulcers on endoscopy, that has a wide differential diagnosis, making the diagnosis even more difficult. The biopsies are falsely negative, as the disease remains mostly in the submucosa and muscularis propria.[5]
- Although the lower mucosal and submucosal layers are mostly involved, the muscular and subserosal layers may also be infiltrated with the neoplastic cells.[7]
Gallery
-
![A signet ring cell carcinoma of the stomach. Signet ring cells are seen in the lower half of the image. Gastric epithelium is seen in the upper half of the image. H&E stain.[8]](/images/3/39/Gastric_signet_ring_cell_carcinoma.jpg)
A signet ring cell carcinoma of the stomach. Signet ring cells are seen in the lower half of the image. Gastric epithelium is seen in the upper half of the image. H&E stain.[8]
-
![Gastric signet ring cell carcinoma. H&E stain.[9]](/images/4/44/Gastric_signet_ring_cell_carcinomahistopatholgy.jpg)
Gastric signet ring cell carcinoma. H&E stain.[9]
![A signet ring cell carcinoma of the stomach. Signet ring cells are seen in the lower half of the image. Gastric epithelium is seen in the upper half of the image. H&E stain.[8]](/index.php/File:Gastric_signet_ring_cell_carcinoma.jpg)
![Gastric signet ring cell carcinoma. H&E stain.[9]](/index.php/File:Gastric_signet_ring_cell_carcinomahistopatholgy.jpg)
References
- ↑ 1.0 1.1 1.2 Linitis plastica. Wikipedia. https://en.wikipedia.org/wiki/Linitis_plastica Accessed on November 18, 2015.
- ↑ 2.0 2.1 2.2 2.3 Gastric linitis plastica. Orphanet. http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=36273 Accessed on December 8, 2015.
- ↑ 3.0 3.1 Wolf, E.-M.; Geigl, J.B.; Svrcek, M.; Vieth, M.; Langner, C. (2010). "Hereditäres Magenkarzinom". Der Pathologe. 31 (6): 423–429. doi:10.1007/s00292-010-1353-7. ISSN 0172-8113.
- ↑ Linitis Plastica. Wikimedia. https://en.wikipedia.org/wiki/Linitis_plastica#/media/File:Linitis_plastica_2.jpg Accessed on December 13, 2015
- ↑ 5.0 5.1 Schauer, M; Peiper, M; Theisen, J; Knoefel, W (2011). "Prognostic factors in patients with diffuse type gastric cancer (linitis plastica) after operative treatment". European Journal of Medical Research. 16 (1): 29. doi:10.1186/2047-783X-16-1-29. ISSN 2047-783X.
- ↑ Stomach cancer. Wikipedia. https://en.wikipedia.org/wiki/Stomach_cancer Accessed on November 19, 2015.
- ↑ Bing Hu, Nassim El Hajj, Scott Sittler, Nancy Lammert, Robert Barnes & Aurelia Meloni-Ehrig (2012). "Gastric cancer: Classification, histology and application of molecular pathology". Journal of gastrointestinal oncology. 3 (3): 251–261. doi:10.3978/j.issn.2078-6891.2012.021. PMID 22943016. Unknown parameter
|month=ignored (help) - ↑ Linitis Plastica. Wikimedia. https://en.wikipedia.org/wiki/Signet_ring_cell_carcinoma#/media/File:Signet_ring_cell_carcinoma_-_very_high_mag.jpg Accessed on December 13, 2015
- ↑ Linitis Plastica. Wikimedia.https://en.wikipedia.org/wiki/Signet_ring_cell_carcinoma#/media/File:Gastric_signet_ring_cell_carcinoma_histopatholgy_%281%29.jpg Accessed on December 13, 2015.
