Pseudomyxoma peritonei pathophysiology: Difference between revisions

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{{Pseudomyxoma peritonei}}
{{Pseudomyxoma peritonei}}
{{CMG}}{{AE}}{{Nnasiri}}{{PSD}}
{{CMG}}{{AE}}{{Nnasiri}}
==Overview==
==Overview==
The remarkable feature of pseudomyxoma peritonei is that this neoplastic, progressive process often arises from a seemingly benign or well differentiated primary tumor. Pseudomyxoma peritonei may be divided into two pathological subtypes which have aetiological and prognostic significance ( Peritoneal adenomucinosis and Peritoneal mucinous carcinoma).
Pseudomyxoma peritonei (PMP) is a rare [[tumor]] known for its production of abundant [[mucinous]] [[ascites]] in the [[abdominal cavity]]. It can have a mass impact on vital organs such as [[spleen]], [[pancreas]], and [[kidney]]. Pseudomyxoma peritonei may be divided into two [[pathological]] subtypes: [[peritoneal]] adenomucinosis and [[Peritoneal mucinous carcinomatosis|peritoneal mucinous carcinoma.]]
 
==Pathogenesis==
==Pathogenesis==
The pathogenesis of the disease is related to biomarkers and molecular genetic alterations.  
The pathogenesis of the disease is related to [[biomarkers]] and molecular [[genetic]] alterations.  
*Immunohistochemical markers involved in the pathogenesis of pseudomyxoma peritonei include:<ref name="pmid10362811">{{cite journal |vauthors=Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM |title=Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women |journal=Am. J. Pathol. |volume=154 |issue=6 |pages=1849–55 |date=June 1999 |pmid=10362811 |pmc=1866622 |doi=10.1016/S0002-9440(10)65442-9 |url=}}</ref>
*[[Immunohistochemistry|Immunohistochemica]]<nowiki/>[[Immunohistochemistry|l markers]] and [[genetic]] alterations involved in the [[pathogenesis]] of pseudomyxoma peritonei include:<ref name="pmid10362811">{{cite journal |vauthors=Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM |title=Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women |journal=Am. J. Pathol. |volume=154 |issue=6 |pages=1849–55 |date=June 1999 |pmid=10362811 |pmc=1866622 |doi=10.1016/S0002-9440(10)65442-9 |url=}}</ref>
**CK 20  
**CK 20  
**CDX2 and MUC2 are found to be positive in these tumors.  
**[[CDX2]] and [[MUC2]] are found to be positive in these [[Tumor|tumors]].  
**KRAS mutation and loss of heterozygosity in some gene loci.
**[[KRAS]] [[Mutations|mutation]] and [[loss of heterozygosity]] in some [[Loci|gene loci]].
**Losses of alleles in chromosomes 18q, 17p, 5q.
**Losses of [[alleles]] in [[chromosomes]] [[18q syndrome|18q]], 17p, 5q.


==Pathology==
==Pathology==
* The remarkable feature of pseudomyxoma peritonei is that this neoplastic, progressive process often arises from a seemingly benign or well differentiated primary tumor.
Pseudomyxoma peritonei may be divided into two pathological subtypes:<ref name="pmid7503361">{{cite journal |vauthors=Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM |title=Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to "pseudomyxoma peritonei" |journal=Am. J. Surg. Pathol. |volume=19 |issue=12 |pages=1390–408 |date=December 1995 |pmid=7503361 |doi= |url=}}</ref>
* Pseudomyxoma peritonei may be divided into two pathological subtypes which have aetiological and prognostic significance:
*[[Disseminated peritoneal adenomucinosis]] (DPAM) which has charracteristic features of the following:
** Peritoneal adenomucinosis
**[[Peritoneal]] lesions with abundant [[extracellular]] [[mucin]]
** Peritoneal mucinous carcinoma
**Proliferative [[mucinous]] [[epithelium]]
* Not all cases may exactly fit into these categories where many of the patients have intermediate or discordant features.
**Less [[mitotic]] activity as compared with [[Peritoneum|peritoneal]] [[mucinous]] [[carcinomatosis]]
 
*[[Peritoneal mucinous carcinomatosis]] (PMCA) which is characterized by:
===Peritoneal adenomucinosis===
**[[Peritoneal]] lesions having more abundant [[mucinous]] [[epithelium]]
*A peritoneal neoplasm composed largely of mucin associated with fibrosis with minimal cytologic atypia and mitoses.
**Characteristic [[Cytological|cytologic]] features of [[carcinoma]], with high [[Mitotic|mitotic activity]].
*The primary tumor is generally an adenoma.
 
===Peritoneal mucinous carcinoma===
*A peritoneal neoplasm characterised by proliferative epithelium, glands, nests, or individual cells with marked cytologic atypia.
*The primary tumor is a mucinous adenocarcinoma.


==Immunohistology==
==Immunohistology==
Immunohistochemical markers can help identify the organ of origin. These include positive cytokeratin 20 (CK20), CEA, caudal-type homeobox protein 2 (CDX-2), and MUC2 as well as negative cytokeratin 7 (CK7) and CA125. Of particular interest is the secreted mucin MUC2 that is extensively positive in pseudomyxoma peritonei patients. Although MUC2 has been suggested as a biological marker of pseudomyxoma peritonei, its significance as a prognostic factor is a matter of controversy.
[[Immunohistochemistry|Immunohistochemical markers]] can help identify the organ of origin.  
*The tumor is positive for [[cytokeratin]] 20 (CK20), [[CEA]], caudal-type homeobox protein 2 (CDX-2), and [[MUC2]] as well as negative [[cytokeratin]] 7 (CK7) and [[CA125]].  
*Studies have shown that mucin [[MUC2]] and MUC5AC is extensively positive in pseudomyxoma peritonei patients. <ref name="pmid16978201">{{cite journal |vauthors=Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M |title=CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases |journal=Histopathology |volume=49 |issue=4 |pages=381–7 |date=October 2006 |pmid=16978201 |doi=10.1111/j.1365-2559.2006.02512.x |url=}}</ref>


== References ==
== References ==
{{Reflist|1}}
{{Reflist|2}}


[[Category:Types of cancer]]
[[Category:Types of cancer]]

Latest revision as of 20:30, 1 April 2019

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

Overview

Pseudomyxoma peritonei (PMP) is a rare tumor known for its production of abundant mucinous ascites in the abdominal cavity. It can have a mass impact on vital organs such as spleen, pancreas, and kidney. Pseudomyxoma peritonei may be divided into two pathological subtypes: peritoneal adenomucinosis and peritoneal mucinous carcinoma.

Pathogenesis

The pathogenesis of the disease is related to biomarkers and molecular genetic alterations.

Pathology

Pseudomyxoma peritonei may be divided into two pathological subtypes:[2]

Immunohistology

Immunohistochemical markers can help identify the organ of origin.

  • The tumor is positive for cytokeratin 20 (CK20), CEA, caudal-type homeobox protein 2 (CDX-2), and MUC2 as well as negative cytokeratin 7 (CK7) and CA125.
  • Studies have shown that mucin MUC2 and MUC5AC is extensively positive in pseudomyxoma peritonei patients. [3]

References

  1. Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM (June 1999). "Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women". Am. J. Pathol. 154 (6): 1849–55. doi:10.1016/S0002-9440(10)65442-9. PMC 1866622. PMID 10362811.
  2. Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM (December 1995). "Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to "pseudomyxoma peritonei"". Am. J. Surg. Pathol. 19 (12): 1390–408. PMID 7503361.
  3. Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M (October 2006). "CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases". Histopathology. 49 (4): 381–7. doi:10.1111/j.1365-2559.2006.02512.x. PMID 16978201.


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