Pediatric Chordomas: A Population-Based Clinical Outcome Study Involving 86 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (1973-2011)

• Published in: Pediatric neurosurgery Vol. 51; no. 3; p. 127
• Main Authors: Lau, Christine S M, Mahendraraj, Krishnaraj, Ward, Amanda, Chamberlain, Ronald S
• Format: Journal Article
• Language: English
• Published: Switzerland 01.01.2016
• Subjects: Adolescent; Adult; Aged; Child; Chordoma - diagnosis; Chordoma - epidemiology; Chordoma - mortality; Cohort Studies; Databases, Factual - trends; Female; Humans; Male; Middle Aged; Population Surveillance - methods; Spinal Neoplasms - diagnosis; Spinal Neoplasms - epidemiology; Spinal Neoplasms - mortality; Survival Rate - trends; Treatment Outcome; Young Adult
• ISSN: 1423-0305
• DOI: 10.1159/000442990

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. 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). 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). 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.