Oncology/endocrine
Improved survival of patients with pseudomyxoma peritonei receiving intraperitoneal chemotherapy with cytoreductive surgery: a systematic review and meta-analysis

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Abstract

Background

Pseudomyxoma peritonei (PMP) is an uncommon but lethal variant of adenocarcinoma. Many recent case series have reported improved survival with the combination of cytoreductive surgery and intraperitoneal chemotherapy (IPEC) in treating PMP. The aim of this study was to analyze the published studies for improved survival with this treatment strategy.

Methods

Data from all studies using IPEC in treating PMP were analyzed. We searched PubMed, MEDLINE, and the Cochrane Library (through September 2011). Studies were limited to English and PMP with appendiceal origin. Twenty-nine studies were identified, with 15 studies from different treatment centers that were specifically analyzed for differences in 5-y mortality and morbidity. Observed to expected (OE) ratios were calculated for both mortality and morbidity.

Results

Mean and median 3-y, 5-y, and 10-y survival rates were 77.18%/77.85%, 76.63%/79.5%, and 57.3%/55.9%, respectively. Of the 10 studies that had sufficient data to calculate OE ratios from the 5-year mortality data, two had OE ratios lower than 1. Of the 11 studies that had data sufficient to calculate OE ratios from the morbidity data, four had OE ratios that were less than 1.

Conclusions

Combining cytoreductive surgery and IPEC improves the survival of patients with PMP, regardless of treatment modality. Although this treatment strategy is associated with an increased risk of morbidity, the increase in survival may be acceptable in proposing an alternative to debulking procedures alone.

Section snippets

Background

Pseudomyxoma peritonei (PMP) is a rare intraperitoneal tumor that originates primarily from appendiceal epithelial neoplasms, usually from ruptured low-grade appendiceal mucinous neoplams [1], [2], [3], [4]. The primary tumor enlarges, leading to obstruction of the appendiceal lumen, which results in perforation of the appendix and dissemination of the tumor within the peritoneal cavity [3], [5]. The disseminated neoplastic cells produce mucin, which leads to the characteristic mucinous ascites

Literature search strategy

The literature was searched using the key terms “pseudomyxoma peritonei” and “intraperitoneal chemotherapy” through September 30, 2011, using the databases of PubMed, OVID MEDLINE, and the Cochrane Library. Studies were limited journal articles that were in the English language, published within the last 20 y to exclude articles published before the use of IPEC.

Exclusion criteria

Case reports, review articles, and abstracts were excluded, as were studies were IPEC was not used, studies which had insufficient

Results

Fifteen studies that reported data on 1624 patients were identified (Table 1). Nine different regimens for HIPEC were used. Five different regimens of EPIC were used, although insufficient data was reported for examining survival rates of two of these regimens (regimens 4 and 5).

The mean mortality rate for the 14 of the 15 studies was 3.75 (one study did not report morbidity and mortality), with a median mortality of 2.45. The mean and median morbidity were 35.75 and 40, respectively. Mean

Discussion

There have been very few clinical trials documenting the use of IPEC and cytoreductive surgery for the treatment of PMP and most of the data on this treatment modality comes from case reports and case series. The lack of data does make an extensive meta-analysis difficult, especially since there is no way to control for the differences in patients, treatment regimens, etc. However, the data that was collected from the studies that have been reported can provide some beneficial insights into the

Conclusion

PMP is a rare disease; however, it remains a disease that can cause significant morbidity and will ultimately result in death if not treated. Historically, treatments have primarily attempted to reduce tumor burden but patients often had to undergo multiple surgeries and the vast majority of patients recurred within 2 years of their first surgery. Treating PMP with a combination of cytoreductive surgery and IPEC can greatly improve survival compared to the natural history of the disease and

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