Primary retroperitoneal XXXX (PRMCa): a systematic review of the literature and meta-analysis.

@entity5 @entity1 @entity4347 @entity490
PURPOSE: Primary retroperitoneal @entity4347 (PRMCa) is a rare @entity5 . 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. METHOD: Systematic review of medical electronic databases for published data (1950-12/10/2015). No RCTs identified. Individual @entity1 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 @entity1 being significantly younger than @entity1 at diagnosis (42.0  years versus 62.2  years, p  =  0.005). A palpable abdominal mass and @entity490 were the most common presentations in 42.9 and 23.8  % of cases, respectively. Twenty-six @entity1 were <38  years old. There were 16 @entity1 <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) @entity1 . 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 @entity1 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. CONCLUSION: PRMCa are rare and potentially aggressive @entity5 that often occur in young @entity1 . @entity5 , adequate staging and adjuvant chemotherapy needs to be considered.
