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 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
- 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
- Peritoneal mucinous carcinoma
- Not all cases may exactly fit into these categories where many of the patients have intermediate or discordant features.
Peritoneal adenomucinosis
- A peritoneal neoplasm composed largely of mucin associated with fibrosis with minimal cytologic atypia and mitoses.
- 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
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.