title: The management of retroperitoneal soft tissue sarcoma: a single institution experience with a review of the literature.
abstract: OBJECTIVE
  Ten percent of soft tissue sarcomas (STS) arise in the retroperitoneal tissues. The prognosis for patients with retroperitoneal sarcoma is poor with a 5-year survival rate between 12% and 70%. Stage at presentation, high histological grade, unresectable primary tumour and incomplete resection are associated with a less favourable outcome.
  
  
  METHODS
  Complete follow-up data were available on 22 patients who underwent surgery for retroperitoneal STS in our institution between 1990 and 2000. Patient, tumour and treatment variables were analysed including use of adjuvant therapy and survival status.
  
  
  RESULTS
  Eighteen patients underwent surgery for primary disease, four patients were treated for recurrent disease or metastases. Ten patients presented with pain, seven with an abdominal mass, other presentation included weight loss and haematuria. Thirteen patients presented with tumours larger than 10 cm. The tumours were seven liposarcomas, six leiomyosarcomas, three malignant fibrous histiocytomas, two rhabdomyosarcomas, two malignant schwannomas and two undifferentiated sarcomas. Six primary tumours were completely excised, five patients received radiotherapy and five received chemotherapy. Local recurrence rate was 45% and recurrence-free interval for 10 patients with recurrence was 11 months. Five patients received radiotherapy and five received chemotherapy. The median survival for patients with primary tumours was 36 months, and 5-year survival was 44%. Adjuvant therapy was not associated with higher survival rates.
  
  
  CONCLUSIONS
  This study re-emphasizes the poor outcome of patients with retroperitoneal STS. Adjuvant radiotherapy and chemotherapy do not appear to be any proven benefit and the single most important prognostic factor is aggressive successful en bloc resection of the primary tumour. Our resection rate and 5-year survival rates are comparable with previous reported UK series although lower than large reports from North American centres. This might partly be explained by difficulty in data collection in a retrospective analysis, but may reflect inadequate subspecialization in UK centres.
authors.affiliation: Department of Surgery, Royal Liverpool University Hospital, Prescot St., Liverpool, L7 8XP, UK.
authors.forename: A
authors.initials: A
authors.lastname: Pirayesh
authors.forename: Y
authors.initials: Y
authors.lastname: Chee
authors.forename: T R
authors.initials: TR
authors.lastname: Helliwell
authors.forename: M J
authors.initials: MJ
authors.lastname: Hershman
authors.forename: S J
authors.initials: SJ
authors.lastname: Leinster
authors.forename: M V
authors.initials: MV
authors.lastname: Fordham
authors.forename: G J
authors.initials: GJ
authors.lastname: Poston
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005500:Follow-Up Studies; D018219:Histiocytoma, Benign Fibrous / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D009442:Neurilemmoma / Q000601:surgery; D011379:Prognosis; D012186:Retroperitoneal Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D012189:Retrospective Studies; D012509:Sarcoma / Q000601:surgery; D012983:Soft Tissue Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome