Minireviews
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World Journal of Gastrointestinal Surgery. Aug 27, 2018; 10(5): 49-56
Published online Aug 27, 2018. doi: 10.4240/wjgs.v10.i5.49
Approach to pseudomyxoma peritonei
Syed Ali Rizvi, Wajahat Syed, Ravi Shergill
Syed Ali Rizvi, Wajahat Syed, Undergraduate Medicine, McMaster University, Hamilton, ON L8S 4L8, Canada
Ravi Shergill, Department of Radiology, McMaster University, Hamilton, ON L8S 4L8, Canada
Author contributions: Rizvi SA drafted the initial manuscript, conceived of the idea, and performed analysis and interpretation of the data; Syed W and Shergill R assisted with data analysis and making critical revisions related to important intellectual content of the manuscript.
Conflict-of-interest statement: There is no conflict of interest associated with any of the authors who contributed their efforts in this manuscript.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Syed Ali Rizvi, BSc, Medical Student, Undergraduate Medicine, McMaster University, 1280 Main Street West, Hamilton, ON L8S 4L8, Canada. ali.rizvi@medportal.ca
Telephone: +1-647-7722061
Received: June 27, 2018
Peer-review started: June 30, 2018
First decision: July 8, 2018
Revised: August 1, 2018
Accepted: August 6, 2018
Article in press: August 6, 2018
Published online: August 27, 2018
Processing time: 62 Days and 19.3 Hours
Abstract

Pseudomyxoma peritonei (PMP) is a mucinous tumour of the appendix that spreads into the peritoneal cavity in the form of gelatinous deposits. The incidence of PMP is believed to be approximately 1-3 out of a million per year. Nonetheless, due to its indolent nature, it is usually discovered at an advanced stage and severely impacts quality of life. Curative treatment for PMP is complete cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). An extensive literature review was conducted searching EMBASE, MEDLINE, PubMed, and Google Scholar databases for PMP in aims to delineate a clinical approach to diagnosis and treatment. Literature was limited to the years 2007-2018. We found the 5-year overall survival with CRS and HIPEC estimated to be between 23%-82% and rates of major complications as high as 24%. Therefore, it is important to appropriately stage and select patients that should undergo CRS with HIPEC. Modalities like MDCT radiological scores have been shown to have sensitivity and specificity of 94% and 81%, respectively, in being able to predict resectability and survival. Despite treatment, the disease often recurs. Tumor markers have significant potential for establishing prognosis pre-operatively, and this paper will review the most recent evidence in support of them.

Keywords: Pseudomyxoma peritonei; Cytoreductive surgery; Hyperthermic intraperitoneal chemotherapy; Mucocele; Appendix

Core tip: This paper highlights the most recent evidence in the clinical approach to pseudomyxoma peritonei. Diagnosis, treatment, complications of treatment, overall survival, and post-operative follow-up will be explored.