Pseudomyxoma peritonei pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]
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
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.