title: Orbital Metastasis Is Associated With Decreased Survival in Stage M Neuroblastoma.
abstract: BACKGROUND
  Approximately 30% of patients with metastatic (stage M) neuroblastoma present with periorbital ecchymosis from orbital osseous disease. Though locoregional disease is staged by imaging, the prognostic significance of metastatic site in stage M disease is unknown. We hypothesize that, compared to nonorbital metastasis, orbital metastasis is associated with decreased survival in patients with stage M neuroblastoma, and that periorbital ecchymosis reflects location and extent of orbital disease.
  
  
  METHODS
  Medical records and imaging from 222 patients with stage M neuroblastoma seen at St. Jude Children's Research Hospital between January 1995 and May 2009 were reviewed. Thirty-seven patients were <18 months of age at diagnosis and 185 were ≥18 months of age. Overall survival (OS) and 5-year survival (5YS) were compared for patients with and without orbital, calvarial and nonorbital osseous metastasis, and with and without periorbital ecchymosis (log-rank test). Associations of periorbital ecchymosis with orbital metastasis location/extent were explored (Fisher's exact test, t-test).
  
  
  RESULTS
  In patients ≥18 months of age, only orbital metastasis was associated with decreased 5YS (P = 0.0323) and OS (P = 0.0288). In patients <18 months of age, neither orbital, calvarial, or nonorbital bone metastasis was associated with OS or 5YS. Periorbital ecchymosis was associated with higher number of involved orbital bones (P = 0.0135), but not location or survival.
  
  
  CONCLUSIONS
  In patients ≥ 18 months of age with stage M neuroblastoma, orbital metastatic disease is associated with decreased 5YS and OS. In future clinical trials, orbital disease may be useful as an imaging-based risk factor for substratification of stage M neuroblastoma.
authors.affiliation: Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Julie H
authors.initials: JH
authors.lastname: Harreld
authors.affiliation: Texas Oculoplastic Consultants, Austin, Texas.
authors.forename: Emily M
authors.initials: EM
authors.lastname: Bratton
authors.affiliation: Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Sara M
authors.initials: SM
authors.lastname: Federico
authors.affiliation: Department of Biostatistics, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Xingyu
authors.initials: X
authors.lastname: Li
authors.affiliation: Department of Ophthalmology, Medstar Georgetown University Hospital, Washington, District of Columbia.
authors.forename: William
authors.initials: W
authors.lastname: Grover
authors.affiliation: Department of Biostatistics, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Yimei
authors.initials: Y
authors.lastname: Li
authors.affiliation: Department of Ophthalmology, University of TN Hamilton Eye Institute, Memphis, Tennessee.|Department of Pediatrics, University of TN College of Medicine, Memphis, Tennessee.|Department of Surgery, Division of Ophthalmology, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Natalie C
authors.initials: NC
authors.lastname: Kerr
authors.affiliation: Department of Ophthalmology, University of TN Hamilton Eye Institute, Memphis, Tennessee.|Department of Surgery, Division of Ophthalmology, St. Jude Children's Research Hospital, Memphis, Tennessee.|Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Matthew W
authors.initials: MW
authors.lastname: Wilson
authors.affiliation: Department of Ophthalmology, University of TN Hamilton Eye Institute, Memphis, Tennessee.|Department of Pediatrics, University of TN College of Medicine, Memphis, Tennessee.|Department of Surgery, Division of Ophthalmology, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Mary E
authors.initials: ME
authors.lastname: Hoehn
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004438:Ecchymosis; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000401:mortality / Q000556:secondary*; D009918:Orbital Neoplasms / Q000401:mortality / Q000556:secondary*; D016016:Proportional Hazards Models
keywords: metastasis; neuroblastoma; orbit; stage M; survival