title: An evidence-based medicine model for rare and often neglected neoplastic conditions.
abstract: OBJECTIVE
  The National Institutes of Health recommends strategies to obtain evidence for the treatment of rare conditions such as primary tumors of the spine (PTSs). These tumors have a low incidence and are pathologically heterogeneous, and treatment approaches are diverse. Appropriate evidence-based care is imperative. Failure to follow validated oncological principles may lead to unnecessary mortality and profound morbidity. This paper outlines a scientific model that provides significant evidence guiding the treatment of PTSs.
  
  
  METHODS
  A four-stage approach was used: 1) planning: data from large-volume centers were reviewed to provide insight; 2) recruitment: centers were enrolled and provided the necessary infrastructure; 3) retrospective stage: existing medical records were reviewed and completed with survival data; and 4) prospective stage: prospective data collection has been implemented. The AOSpine Knowledge Forum Tumor designed six modules: demographic, clinical, diagnostic, therapeutic, local recurrence, survival, and perioperative morbidity data fields and provided funding.
  
  
  RESULTS
  It took 18 months to implement Stages 1-3, while Stage 4 is ongoing. A total of 1495 tumor cases were captured and diagnosed as one of 18 PTS histotypes. In addition, a PTS biobank network has been created to link clinical data with tumor pathology and molecular analysis.
  
  
  CONCLUSIONS
  This scientific model has not only aggregated a large amount of PTS data, but has also established an international collaborative network of spine oncology centers. Access to large volumes of data will generate further research to guide and enhance PTS clinical management. This model could be applied to other rare neoplastic conditions. Clinical trial registration no.: NCT01643174 (ClinicalTrials.gov).
authors.affiliation: Division of Spine, Department of Orthopaedics, University of British Columbia and Vancouver CoastalHealth, Vancouver, British Columbia;
authors.forename: Charles G
authors.initials: CG
authors.lastname: Fisher
authors.forename: Tony
authors.initials: T
authors.lastname: Goldschlager
authors.forename: Stefano
authors.initials: S
authors.lastname: Boriani
authors.forename: Peter Paul
authors.initials: PP
authors.lastname: Varga
authors.forename: Laurence D
authors.initials: LD
authors.lastname: Rhines
authors.forename: Michael G
authors.initials: MG
authors.lastname: Fehlings
authors.forename: Alessandro
authors.initials: A
authors.lastname: Luzzati
authors.forename: Mark B
authors.initials: MB
authors.lastname: Dekutoski
authors.forename: Jeremy J
authors.initials: JJ
authors.lastname: Reynolds
authors.forename: Dean
authors.initials: D
authors.lastname: Chou
authors.forename: Sigurd H
authors.initials: SH
authors.lastname: Berven
authors.forename: Richard P
authors.initials: RP
authors.lastname: Williams
authors.forename: Nasir A
authors.initials: NA
authors.lastname: Quraishi
authors.forename: Chetan
authors.initials: C
authors.lastname: Bettegowda
authors.forename: Ziya L
authors.initials: ZL
authors.lastname: Gokaslan
mesh_terms: D000328:Adult; D003430:Cross-Sectional Studies; D019317:Evidence-Based Medicine; D005260:Female; D014943:Global Health; D006801:Humans; D015994:Incidence; D008297:Male; D019635:Neurosurgical Procedures; D011446:Prospective Studies; D035583:Rare Diseases / Q000453:epidemiology / Q000473:pathology / Q000601:surgery*; D012189:Retrospective Studies; D013125:Spinal Neoplasms / Q000453:epidemiology / Q000473:pathology / Q000601:surgery*; D011795:Surveys and Questionnaires; D015996:Survival Rate; D014481:United States
keywords: AOSKFT = AOSpine International Knowledge Forum Tumor; FFPE = formalin-fixed paraffinembedded; NIH = National Institutes of Health; PTS = primary tumor of the spine; WBB = Weinstein-Boriani-Biagini; network; oncology; primary tumor; rare disease; scientific model; spine; surgery