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Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011).
- Source :
-
Pediatric neurosurgery [Pediatr Neurosurg] 2016; Vol. 51 (3), pp. 127-36. Date of Electronic Publication: 2016 Feb 17. - Publication Year :
- 2016
-
Abstract
- Background/aims: 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.<br />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).<br />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).<br />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.<br /> (© 2016 S. Karger AG, Basel.)
- Subjects :
- Adolescent
Adult
Aged
Child
Chordoma diagnosis
Chordoma epidemiology
Cohort Studies
Female
Humans
Male
Middle Aged
Spinal Neoplasms diagnosis
Spinal Neoplasms epidemiology
Survival Rate trends
Treatment Outcome
Young Adult
Chordoma mortality
Databases, Factual trends
Population Surveillance methods
Spinal Neoplasms mortality
Subjects
Details
- Language :
- English
- ISSN :
- 1423-0305
- Volume :
- 51
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Pediatric neurosurgery
- Publication Type :
- Academic Journal
- Accession number :
- 26881831
- Full Text :
- https://doi.org/10.1159/000442990