Treatment was essentially restricted to interval debulking with no realistic prospect of long-term cure 6. In the past, this disease was uniformly fatal. Tumor markers can be used to monitor response to treatment and as surveillance tools to look for recurrence. They reveal a correlation between a normal preoperative CA-125 with the likelihood of achieving adequate cytoreduction at surgery (a significant prognostic factor for PMP) and go on to suggest that an increased baseline CA19.9 is an independent inverse predictor of progression-free survival after treatment 15. however demonstrate a role for these markers in post-treatment prognostication. Tumor markers CEA, CA-19.9, and CA-125 are raised, the specificity and sensitivity of these markers in PMP has not been extensively studied and therefore their diagnostic utility in PMP is likely to be restricted at best. The role of MR imaging in PMP is, however, yet to be established 13.Īlthough anaemia is a common finding, laboratory studies are of very limited use in diagnosing PMP, with the definitive diagnosis usually being established at laparotomy 14. argue that due to high water content, mucin has a similar appearance to water on both CT and MR imaging, but when the mucin produced is thick and proteinaceous then it tends to be hyperdense compared to water and hyperintense on T1- and hypointense on T2-weighted images. Septa, curvilinear or amorphous calcification, areas of soft tissue attenuation due to solid elements within mucinous material and compressed mesentery are seen as the volume of disease increases 12. In most cases, the striking feature is the relative sparing of the small bowel and its mesentery, with the small bowel “compartmentalized” in the center of the abdomen by a large omental cake 11. The imaging and staging modality of choice is abdominal CT scanning 10, with scalloping and characteristic patterns of disease on visceral surfaces being almost pathognomic of PMP. Other classification systems exist but the one devised by Ronnett et al. DPAM, a low-grade lesion with a good prognosis peritoneal mucinous adenocarcinoma (PMCA), which is histopathologically a high-grade metastatic adenocarcinoma, usually derived from the appendix and colon, with a poor prognosis and intermediate type PMP (PMCA-I), which includes lesions that demonstrate predominantly features of DPAM, but also contain focal areas of PMCA (intermediate prognosis) 9. proposed three pathological subtypes of the condition 8. The locoregional progression of the disease (redistribution phenomenon) 6 results in intestinal failure and malnutrition secondary to raised intra-abdominal pressure, fistula formation, and infection 7, hence the associated considerable mortality and morbidity. The disease is characterized by diffuse intra-abdominal gelatinous collections (jelly belly) with mucinous implants on peritoneal surfaces and the omentum 4. Accumulation and the reproduction of free and implanted tumor cells leads to progressive peritoneal mucinous tumor and ascites 4. ![]() These cells show a high propensity for spread to peritoneal surfaces, but almost no lymphatic or hematogenous metastases 5. Most cases originate from a minimally invasive mucinous epithelial neoplasm (as mentioned above primarily appendiceal but ovarian and colonic sources have also been described), which eventually ruptures and releases mucinous tumor cells into the peritoneal cavity 4. Immunohistochemistry and molecular genetics have, however, lent strength to the current concept of an appendiceal origin for the vast majority of PMP in both men and women 1. Points of contention as to the exact origin of this condition, especially in women, stem from the fact that synchronous appendiceal and ovarian disease is common and the overall female preponderance for PMP 3. Coined by Werth in 1884 2, PMP was initially thought of as a peritoneal reaction to the “jelly-like” secretions produced by an ovarian neoplasm 1. Pseudomyxoma peritonei (PMP) is a rare diagnosis with an incidence of 1–2 per million 1.
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