title: Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011).
abstract: OBJECTIVE
  Primary chordomas, rare cancers arising from the notochord remnants, are extremely rare in the pediatric population. This study examined a large cohort of primary chordoma patients to determine factors impacting prognosis and survival.
  
  
  METHODS
  Demographic and clinical data on 1,358 primary chordoma patients (86 pediatric patients ≤19 years of age and 1,272 adult patients ≥20 years of age) were abstracted from the Surveillance, Epidemiology, and End Result (SEER) database (1973-2011).
  
  
  RESULTS
  Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite the majority of primary chordomas presenting with locoregional involvement (90.4%), pediatric patients had more distant disease (14.8 vs. 9.2%, p < 0.05). Survival among pediatric patients having surgery only was significantly longer than for adults (22.5 vs. 14.3 years, p < 0.001). Overall survival was longer (17.2 vs. 12.6 years) and overall mortality was lower in pediatric patients (38.4 vs. 49.8%), but cancer-specific mortality was higher (37.2 vs. 28.6%, p < 0.005).
  
  
  CONCLUSIONS
  Pediatric primary chordomas present most often as small tumors <4 cm in the cranium of male Caucasians. Despite having a higher rate of metastasis, they have prolonged survival compared to adults. Surgical resection significantly improves survival in pediatric primary chordoma patients, and should be considered as first-line therapy in all eligible children.
authors.affiliation: Department of Surgery, Saint Barnabas Medical Center, Livingston, N.J., USA.
authors.forename: Christine S M
authors.initials: CS
authors.lastname: Lau
authors.forename: Krishnaraj
authors.initials: K
authors.lastname: Mahendraraj
authors.forename: Amanda
authors.initials: A
authors.lastname: Ward
authors.forename: Ronald S
authors.initials: RS
authors.lastname: Chamberlain
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002817:Chordoma / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality*; D015331:Cohort Studies; D016208:Databases, Factual / Q000639:trends*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011159:Population Surveillance / Q000379:methods; D013125:Spinal Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality*; D015996:Survival Rate / Q000639:trends; D016896:Treatment Outcome; D055815:Young Adult