Pseudomyxoma peritonei pathophysiology
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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 composed of abundant extracellular mucin containing scant simple to focally proliferative mucinous epithelium with little cytologic atypia or mitotic activity, with or without an associated appendiceal mucinous adenoma.
- Peritoneal mucinous carcinomatosis (PMCA) which is characterized by peritoneal lesions having more abundant mucinous epithelium with the architectural and cytologic features of carcinoma, with or without an associated primary mucinous adenocarcinoma.
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.
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.