title: 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.
abstract: OBJECTIVE
  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.
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: University of New South Wales, St George Hospital, Sydney, Australia. terence.chua@unsw.edu.au
authors.forename: Terence C
authors.initials: TC
authors.lastname: Chua
authors.forename: Brendan J
authors.initials: BJ
authors.lastname: Moran
authors.forename: Paul H
authors.initials: PH
authors.lastname: Sugarbaker
authors.forename: Edward A
authors.initials: EA
authors.lastname: Levine
authors.forename: Olivier
authors.initials: O
authors.lastname: Glehen
authors.forename: François N
authors.initials: FN
authors.lastname: Gilly
authors.forename: Dario
authors.initials: D
authors.lastname: Baratti
authors.forename: Marcello
authors.initials: M
authors.lastname: Deraco
authors.forename: Dominique
authors.initials: D
authors.lastname: Elias
authors.forename: Armando
authors.initials: A
authors.lastname: Sardi
authors.forename: Winston
authors.initials: W
authors.lastname: Liauw
authors.forename: Tristan D
authors.initials: TD
authors.lastname: Yan
authors.forename: Pedro
authors.initials: P
authors.lastname: Barrios
authors.forename: Alberto
authors.initials: A
authors.lastname: Gómez Portilla
authors.forename: Ignace H J T
authors.initials: IH
authors.lastname: de Hingh
authors.forename: Wim P
authors.initials: WP
authors.lastname: Ceelen
authors.forename: Joerg O
authors.initials: JO
authors.lastname: Pelz
authors.forename: Pompiliu
authors.initials: P
authors.lastname: Piso
authors.forename: Santiago
authors.initials: S
authors.lastname: González-Moreno
authors.forename: Kurt
authors.initials: K
authors.lastname: Van Der Speeten
authors.forename: David L
authors.initials: DL
authors.lastname: Morris
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001063:Appendiceal Neoplasms / Q000473:pathology*; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D006979:Hyperthermia, Induced / Q000379:methods*; D007274:Injections, Intraperitoneal; D008297:Male; D008875:Middle Aged; D010534:Peritoneal Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000556:secondary* / Q000601:surgery / Q000628:therapy*; D011553:Pseudomyxoma Peritonei / Q000188:drug therapy* / Q000401:mortality / Q000601:surgery*; D015996:Survival Rate; D055815:Young Adult