title: Outcomes after resection of leiomyosarcomas of the inferior vena cava: a pooled data analysis of 377 cases.
abstract: BACKGROUND
  Primary leiomyosarcomas of the inferior vena cava (IVC) pose unique surgical challenges. Due to the rarity of the disease, little definitive data exists on prognosis and treatment options.
  
  
  METHODS
  A pooled data analysis was performed on all cases of initial IVC leiomyosarcoma resection identified by literature search (n = 371) and our institutional database (n = 6). Kaplan-Meier and Cox regression analyses were performed to identify factors associated with disease-free survival (DFS) and overall survival (OS).
  
  
  RESULTS
  Patients were predominantly female (76%, n = 286); the median age of presentation was 55 years. Five-year DFS and OS were 6% and 55%, respectively. Preoperative factors independently associated with decreased OS included older age (HR:1.05, 95% CI:1.00-1.09), larger tumor size (HR:1.14, 95% CI:1.04-1.24), resection of adjacent organ(s) (HR:3.62, 95% CI:1.34-9.77), and R2 resection (HR:7.80, 95% CI:1.94-32.05). Isolated involvement of the suprarenal infrahepatic IVC was associated with longer OS (HR:0.22, 95% CI:0.06-0.78). A scoring system incorporating independent predictors of OS stratified outcomes: score 4-5 (n = 10, median OS 6 months), score 2-3 (n = 88, median OS 23 months) compared to a score of 0-1 (n = 44, median OS 29 months).
  
  
  CONCLUSIONS
  Following resection of IVC leiomyosarcomas, recurrence is a near certainty; long-term survival, however is possible. The dominant predictors of survival include margin status, tumor size and radical resection. These can be combined into a risk score that has prognostic value.
authors.affiliation: Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA. Electronic address: Heather.Wachtel@uphs.upenn.edu.
authors.forename: Heather
authors.initials: H
authors.lastname: Wachtel
authors.affiliation: Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Meera
authors.initials: M
authors.lastname: Gupta
authors.affiliation: Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Edmund K
authors.initials: EK
authors.lastname: Bartlett
authors.affiliation: Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Benjamin M
authors.initials: BM
authors.lastname: Jackson
authors.affiliation: Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Rachel R
authors.initials: RR
authors.lastname: Kelz
authors.affiliation: Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Giorgos C
authors.initials: GC
authors.lastname: Karakousis
authors.affiliation: Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Douglas L
authors.initials: DL
authors.lastname: Fraker
authors.affiliation: Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Robert E
authors.initials: RE
authors.lastname: Roses
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007890:Leiomyosarcoma / Q000473:pathology* / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D012044:Regression Analysis; D012307:Risk Factors; D016896:Treatment Outcome; D014682:Vena Cava, Inferior / Q000473:pathology* / Q000601:surgery*
keywords: IVC; Inferior vena cava; Leiomyosarcoma; Sarcoma