Pseudomyxoma peritonei pathophysiology: Difference between revisions
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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). | 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). | ||
==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. | ||
* | *[[Immunohistochemica]]<nowiki/>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 tumors. | ||
**KRAS mutation and loss of heterozygosity in some gene loci. | **[[KRAS]] [[Mutations|mutation]] and [[loss of heterozygosity]] in some gene loci. | ||
**Losses of alleles in chromosomes 18q, 17p, 5q. | **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> | 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> | ||
*Disseminated peritoneal adenomucinosis (DPAM) which characterized by peritoneal lesions with abundant extracellular mucin containing proliferative mucinous epithelium with less mitotic activity compare with peritoneal mucinous carcinomatosis, with or without appendiceal mucinous adenoma. | *[[Disseminated peritoneal adenomucinosis]] (DPAM) which characterized by [[peritoneal]] lesions with abundant extracellular [[mucin]] containing proliferative mucinous epithelium with less [[mitotic]] activity compare with peritoneal mucinous carcinomatosis, with or without appendiceal mucinous adenoma. | ||
*Peritoneal mucinous carcinomatosis (PMCA) which is characterized by peritoneal lesions having more abundant mucinous epithelium with characteristic cytologic features of carcinoma, with or without an associated primary mucinous adenocarcinoma. | *[[Peritoneal mucinous carcinomatosis]] (PMCA) which is characterized by [[peritoneal]] lesions having more abundant mucinous [[epithelium]] with characteristic cytologic features of [[carcinoma]], with or without an associated primary mucinous adenocarcinoma. | ||
==Immunohistology== | ==Immunohistology== | ||
Immunohistochemical markers can help identify the organ of origin. | 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. | *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 | *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 == | ||
Revision as of 15:10, 9 January 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]Parminder Dhingra, M.D. [3]
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).
Pathogenesis
The pathogenesis of the disease is related to biomarkers and molecular genetic alterations.
- Immunohistochemical markers and genetic alterations involved in the pathogenesis of pseudomyxoma peritonei include:[1]
- CK 20
- CDX2 and MUC2 are found to be positive in these tumors.
- KRAS mutation and loss of heterozygosity in some gene loci.
- Losses of alleles in chromosomes 18q, 17p, 5q.
Pathology
Pseudomyxoma peritonei may be divided into two pathological subtypes:[2]
- Disseminated peritoneal adenomucinosis (DPAM) which characterized by peritoneal lesions with abundant extracellular mucin containing proliferative mucinous epithelium with less mitotic activity compare with peritoneal mucinous carcinomatosis, with or without appendiceal mucinous adenoma.
- Peritoneal mucinous carcinomatosis (PMCA) which is characterized by peritoneal lesions having more abundant mucinous epithelium with characteristic cytologic features of carcinoma, with or without an associated primary mucinous adenocarcinoma.
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
- ↑ 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.
- ↑ 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.
- ↑ 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.