Pseudomyxoma peritonei pathophysiology: Difference between revisions

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{{Pseudomyxoma peritonei}}
{{Pseudomyxoma peritonei}}
{{CMG}}{{AE}}{{PSD}}
{{CMG}}{{AE}}{{Nnasiri}}
==Overview==
==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 carcinomatosis|peritoneal mucinous carcinoma.]]


==Pathophysiology==
==Pathogenesis==
The pathological process starts with neoplastic transformation of the appendiceal goblet cells and subsequent formation of a primary mucinous tumor. While proliferating, tumor cells maintain their constitutive level of mucin expression. As a result, the overall secretion of mucin dramatically rises. This is followed by intraluminal accumulation of mucin and eventual development of an appendiceal mucocele. A small perforation or rupture of the mucocele is the key event towards the development of PMP through which tumor cells gain access into the peritoneal cavity. Lacking cell surface adhesion molecules, the exfoliated tumor cells passively circulate with the peritoneal fluid and redistribute throughout the peritoneal cavity. As a result, tumor implants and mucin collections form at the peritoneal fluid reabsorption sites as well as within the dependent portions of the peritoneal cavity to create PMP’s characteristic pattern of the peritoneal dissemination. Accumulating mucin increases intraabdominal pressure and compresses visceral organs. Furthermore, extensive involvement of the peritoneal surface promotes variable inflammatory and fibrotic responses in the peritoneal environment and hence the development of bowel obstruction as a fatal complication of the disease.<ref name="AminiMasoumi-Moghaddam2014">{{cite journal|last1=Amini|first1=Afshin|last2=Masoumi-Moghaddam|first2=Samar|last3=Ehteda|first3=Anahid|last4=Morris|first4=David|title=Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects|journal=Orphanet Journal of Rare Diseases|volume=9|issue=1|year=2014|pages=71|issn=1750-1172|doi=10.1186/1750-1172-9-71}}</ref>
The pathogenesis of the disease is related to [[biomarkers]] and molecular [[genetic]] alterations.  
*[[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
**[[CDX2]] and [[MUC2]] are found to be positive in these [[Tumor|tumors]].
**[[KRAS]] [[Mutations|mutation]] and [[loss of heterozygosity]] in some [[Loci|gene loci]].
**Losses of [[alleles]] in [[chromosomes]] [[18q syndrome|18q]], 17p, 5q.


==Pathology==
==Pathology==
 
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>
The remarkable feature of pseudomyxoma peritonei is that this neoplastic, progressive process often arises from a seemingly benign or well differentiated primary tumor.
*[[Disseminated peritoneal adenomucinosis]] (DPAM) which has charracteristic features of the following:
 
**[[Peritoneal]] lesions with abundant [[extracellular]] [[mucin]]
Pseudomyxoma peritonei may be divided into two pathological subtypes which have aetiological and prognostic significance:
**Proliferative [[mucinous]] [[epithelium]]
 
**Less [[mitotic]] activity as compared with [[Peritoneum|peritoneal]] [[mucinous]] [[carcinomatosis]]
===Peritoneal adenomucinosis===
*[[Peritoneal mucinous carcinomatosis]] (PMCA) which is characterized by:
A peritoneal neoplasm composed largely of mucin associated with fibrosis with minimal cytologic atypia and mitoses
**[[Peritoneal]] lesions having more abundant [[mucinous]] [[epithelium]]
The primary tumor is generally an adenoma
**Characteristic [[Cytological|cytologic]] features of [[carcinoma]], with high [[Mitotic|mitotic activity]].
 
===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
 
Not all cases fit neatly into these categories, and many patients have intermediate or discordant features.


==Immunohistology==
==Immunohistology==
Immunohistochemical markers can help to 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 the patients. Although MUC2 has been suggested as a biological marker of PMP, 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 ==
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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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