title: Primary retroperitoneal mucinous cystadenocarcinoma (PRMCa): a systematic review of the literature and meta-analysis.
abstract: OBJECTIVE
  Primary retroperitoneal mucinous cystadenocarcinoma (PRMCa) is a rare tumour. Prognosis and optimal management are not well established. In view of a case managed in our Centre, we performed a systematic review and meta-analysis.
  
  
  METHODS
  Systematic review of medical electronic databases for published data (1950-12/10/2015). No RCTs identified. Individual patient data detracted from case reports and case series were analysed
  
  
  RESULTS
  In total, 73 female and 5 male cases of PRMCa identified including our case. Median age at diagnosis was 42.0 years (range 18-86 years), with women being significantly younger than men at diagnosis (42.0 years versus 62.2 years, p = 0.005). A palpable abdominal mass and abdominal pain were the most common presentations in 42.9 and 23.8 % of cases, respectively. Twenty-six women were <38 years old. There were 16 women <38 years old that had surgical data reported, of which 14 underwent fertility-sparing surgery with excision of the mass. Adjuvant chemotherapy was given in 24.1 % (13/72) women. Follow-up ranged from 1 to 130 months with a median of 15 months. Of the 57 cases that had follow-up reported, recurrence occurred in 23 cases (40.4 %) within a median of 8 months from diagnosis. Median disease-free survival was 15 months (range 1-130 months). Of the women who recurred, 14 died of their disease giving 1, 2 and 5-year disease-specific survival rates of 85.9, 80.7 and 75.4 %, respectively.
  
  
  CONCLUSIONS
  PRMCa are rare and potentially aggressive tumours that often occur in young women. Removal of the tumour, adequate staging and adjuvant chemotherapy needs to be considered.
authors.affiliation: Department of Gynaecological Oncology, University Hospitals of Leicester, Leicester, UK. evamyriokefalitaki@mycosmos.gr.
authors.forename: E
authors.initials: E
authors.lastname: Myriokefalitaki
authors.affiliation: Department of Gynaecological Oncology, University Hospitals of Leicester, Leicester, UK.
authors.forename: I
authors.initials: I
authors.lastname: Luqman
authors.affiliation: Department of Gynaecological Oncology, University Hospitals of Leicester, Leicester, UK.
authors.forename: N
authors.initials: N
authors.lastname: Potdar
authors.affiliation: Department of Pathology, University Hospitals of Leicester, Leicester, UK.
authors.forename: L
authors.initials: L
authors.lastname: Brown
authors.affiliation: Department of Cancer Studies and Molecular Medicine, University of Leicester, Leicester Royal Infirmary, Leicester, LE2 7LX, UK.
authors.forename: W
authors.initials: W
authors.lastname: Steward
authors.affiliation: Department of Gynaecological Oncology, University Hospitals of Leicester, Leicester, UK.|Department of Cancer Studies and Molecular Medicine, University of Leicester, Leicester Royal Infirmary, Leicester, LE2 7LX, UK.
authors.forename: E L
authors.initials: EL
authors.lastname: Moss
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017024:Chemotherapy, Adjuvant / Q000379:methods; D018282:Cystadenocarcinoma, Mucinous / Q000401:mortality* / Q000601:surgery*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D011379:Prognosis; D012186:Retroperitoneal Neoplasms / Q000401:mortality* / Q000601:surgery*; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult
keywords: Mucinous cystadenocarcinoma; Primary; Prognosis; Retroperitoneal; Treatment