title: Primary Ewing tumor of the vertebrae: clinical characteristics, prognostic factors, and outcome.
abstract: BACKGROUND
  Fewer than 10% of Ewing family of tumors (EFT) arise in the vertebrae. Little information is available regarding the clinical presentation and outcome of these tumors.
  
  
  METHODS
  We reviewed the clinical features, prognostic factors, and outcome of EFT of the spine identified at our institution between 1962 and 1999.
  
  
  RESULTS
  Thirty-three (10%) of 344 patients with EFT had a primary vertebral tumor. There were 21 (64%) males. Median age at diagnosis was 13.3 years. Six patients had metastatic disease and 10 had tumors > or = 8 cm in diameter. Primary sites were sacral (13), thoracic (10), lumbar (8), and cervical (2) vertebrae. We found no association between the affected spinal region and outcome, although sacral tumors were associated with delayed diagnosis (4 vs. 2 months after onset of symptoms, P = 0.076). Pain (n = 32) and neurologic deficits (n = 31; 82% motor, 58% sensory, 42% bladder, 27% bowel) were the most common presenting features. All patients received combination chemotherapy and local radiotherapy. With a median follow up of 9.7 years, 5-year survival and event-free survival ( +/- SD) estimates were 48.1% (8.9%) and 35.6% (8.6%), respectively, comparable to those of other patients with EFT. Outcome was better for patients with tumor size < 8 cm (P = 0.008) or localized disease (P = 0.084). Treatment era and specific tumor site did not affect outcome.
  
  
  CONCLUSIONS
  Outcomes are similar for primary EFT of the spine and primary EFT in other sites. Unlike others, we found that patients with sacral tumors did not fare worse than patients with tumors at other spinal sites.
authors.affiliation: Department of Hematology-Oncology, St. Jude Children's Research Hospital Memphis, Tennessee 38105-2794, USA.
authors.forename: L
authors.initials: L
authors.lastname: Venkateswaran
authors.forename: C
authors.initials: C
authors.lastname: Rodriguez-Galindo
authors.forename: T E
authors.initials: TE
authors.lastname: Merchant
authors.forename: C A
authors.initials: CA
authors.lastname: Poquette
authors.forename: B N
authors.initials: BN
authors.lastname: Rao
authors.forename: A S
authors.initials: AS
authors.lastname: Pappo
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000556:secondary / Q000628:therapy*; D013125:Spinal Neoplasms / Q000175:diagnosis* / Q000556:secondary / Q000628:therapy*; D016896:Treatment Outcome