Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy

Terence C. Chua*, Brendan J. Moran, Paul H. Sugarbaker, Edward A. Levine, Olivier Glehen, François N. Gilly, Dario Baratti, Marcello Deraco, Dominique Elias, Armando Sardi, Winston Liauw, Tristan D. Yan, Pedro Barrios, Alberto Goḿez Portilla, Ignace H J T De Hingh, Wim P. Ceelen, Joerg O. Pelz, Pompiliu Piso, Santiago Gonzaĺez-Moreno, Kurt Van Der SpeetenDavid L. Morris

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

910 Citations (Scopus)

Abstract

Purpose: Pseudomyxoma peritonei (PMP) originating from an appendiceal mucinous neoplasm remains a biologically heterogeneous disease. The purpose of our study was to evaluate outcome and long-term survival after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) consolidated through an international registry study. Patients and Methods: A retrospective multi-institutional registry was established through collaborative efforts of participating units affiliated with the Peritoneal Surface Oncology Group International. Results: Two thousand two hundred ninety-eight patients from 16 specialized units underwent CRS for PMP. Treatment-related mortality was 2% and major operative complications occurred in 24% of patients. The median survival rate was 196 months (16.3 years) and the median progression-free survival rate was 98 months (8.2 years), with 10- and 15-year survival rates of 63% and 59%, respectively. Multivariate analysis identified prior chemotherapy treatment (P = .001), peritoneal mucinous carcinomatosis (PMCA) histopathologic subtype (P = .001), major postoperative complications (P = .008), high peritoneal cancer index (P = .013), debulking surgery (completeness of cytoreduction [CCR], 2 or 3; P = .001), and not using HIPEC (P = .030) as independent predictors for a poorer progression-free survival. Older age (P = .006), major postoperative complications (P = .001), debulking surgery (CCR 2 or 3; P = .001), prior chemotherapy treatment (P = .001), and PMCA histopathologic subtype (P = .001) were independent predictors of a poorer overall survival. Conclusion: The combined modality strategy for PMP may be performed safely with acceptable morbidity and mortality in a specialized unit setting with 63% of patients surviving beyond 10 years. Minimizing nondefinitive operative and systemic chemotherapy treatments before definitive cytoreduction may facilitate the feasibility and improve the outcome of this therapy to achieve long-term survival. Optimal cytoreduction achieves the best outcomes.

Original languageEnglish
Pages (from-to)2449-2456
Number of pages8
JournalJournal of Clinical Oncology
Volume30
Issue number20
DOIs
Publication statusPublished - 10 Jul 2012
Externally publishedYes

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