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Annals of Surgical Oncology
Published in: Annals of Surgical Oncology 13/2016

01-12-2016 | Gastrointestinal Oncology

Pseudomyxoma Peritonei of Extra-Appendiceal Origin: A Comparative Study

Authors: Dario Baratti, MD, Shigeki Kusamura, MD, PhD, Massimo Milione, MD, Filippo Pietrantonio, MD, Marta Caporale, MD, Marcello Guaglio, MD, Marcello Deraco, MD

Published in: Annals of Surgical Oncology | Issue 13/2016

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Abstract

Background

Pseudomyxoma peritonei (PMP) usually originates from appendiceal neoplasms and, less commonly, from extra-appendiceal lesions. To date, the clinical and therapeutic implications of extra-appendiceal origin are largely unknown.

Methods

A prospective database of 225 PMP patients uniformly treated by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) was reviewed to identify cases with extra-appendiceal primaries. Histologically, negative appendix defined extra-appendiceal origin. Clinical, pathological, and immunohistochemical features (cytokeratin [CK]-20, CK-7, CDX-2, MUC-2, MUC-5A) were correlated with the site of origin. PMP was categorized into low or high grade, according to the 2010 World Health Organization (WHO) classification. The main independent variable for survival analysis was appendiceal versus extra-appendiceal primary.

Results

In 19 patients (8.4 %), PMP origin was the ovary (n = 9), uterine cervix (n = 1), mature cystic teratomas (n = 4), and unknown (n = 5). Appendiceal and extra-appendiceal PMP groups were comparable for all characteristics, except for a prevalence of females in the latter. Median follow-up was 64.1 months (95 % confidence interval [CI] 53.9–80.1), and 10-year overall survival was 63.4 % (median 148.2 months; 95 % CI 131.2–165.2) for appendiceal PMP, and 62.0 % (median not reached) for extra-appendiceal PMP. The difference was not significant at univariate ( p = 0.297) and multivariate analysis (hazard ratio 1.51, 95 % CI 0.78–3.14; p = 0.278). High-grade peritoneal histology (p = 0.007), prior systemic chemotherapy (p = 0.003), more than four visceral resections (p = 0.011), and incomplete cytoreduction (p = 0.021) independently correlated with poorer survival.

Conclusions

Clinical-pathological features of PMP, and outcome after CRS/HIPEC, did not differ according to the primary site, thus suggesting that PMP is a relatively homogeneous disease that can be produced by a range of histopathologic entities. Extra-appendiceal origin does not contraindicate CRS/HIPEC.
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Metadata
Title
Pseudomyxoma Peritonei of Extra-Appendiceal Origin: A Comparative Study
Authors
Dario Baratti, MD
Shigeki Kusamura, MD, PhD
Massimo Milione, MD
Filippo Pietrantonio, MD
Marta Caporale, MD
Marcello Guaglio, MD
Marcello Deraco, MD
Publication date
01-12-2016
Publisher
Springer International Publishing
Published in
Annals of Surgical Oncology / Issue 13/2016
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-016-5350-9

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