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
Pathophysiology
Pseudomyxoma peritonei refers to intraperitoneal accumulation of a gelatinous ascites secondary to rupture of a mucinous tumor. The most common cause is a ruptured mucinous tumor of the appendix / appendiceal mucocoele.
Pseudomyxoma peritonei is commonly discovered during surgery for other conditions, i.e., hernia repair, following which an experienced pathologist can confirm the diagnosis. Due to the rarity of this disease, it is important to obtain an accurate diagnosis so that appropriate treatment may be obtained.
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
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
Not all cases fit neatly into these categories, and many patients have intermediate or discordant features.