title: Primary spinal myxopapillary ependymoma in the pediatric population: a study from the Surveillance, Epidemiology, and End Results (SEER) database.
abstract: Primary spinal myxopapillary ependymomas (MPE) in children are extremely rare. We examined the patient demographics, treatment modalities, and the associated outcomes of children with MPE using the Surveillance, Epidemiology, and End Results (SEER) national cancer database to gain a better understanding of these tumors. The SEER database (1973-2012) was used to analyze patients 21 years of age and younger with histologically confirmed MPE localized to the spinal cord or cauda equina. We analyzed patient demographics, extent of surgical resection, and radiation treatment. Overall survival was calculated using the Kaplan-Meier method. Statistical significance was defined as p < 0.05, with all data analyzed in IBM SPSS Statistics 21. 122 pediatric patients met inclusion criteria. The median age was 16 years (range 0-21) with 63 % male and 87 % Caucasian. The mean follow-up time was 4.5 years (95 % CI 3.93-5.07). Overall survival at 5 and 10 years was 97 and 95 %, respectively. We found 37 % underwent gross-total resection (GTR), 36 % subtotal resection (STR), and 27 % biopsy only. Patients who received GTR alone (n = 37) had a statistically significant increase in overall survival compared to those who received STR plus adjuvant radiation (n = 20) (Χ2= 5.9, p < 0.05). To our knowledge, this is the largest survival analysis of pediatric patients with MPE. Overall survival is excellent at the 5 and 10-year time points; however, GTR should be the goal of treatment when possible. For patients with MPE, future studies should focus on longer follow-up, the role of radiation, and the therapeutic approach at tumor recurrence.
authors.affiliation: Frank H. Netter MD School of Medicine, Quinnipiac University, 370 Bassett Road, North Haven, CT, 06473, USA. kelly.lucchesi@quinnipiac.edu.
authors.forename: Kelly M
authors.initials: KM
authors.lastname: Lucchesi
authors.affiliation: Department of Neurosurgery, School of Medicine, Yale University, New Haven, CT, USA.
authors.forename: Ryan
authors.initials: R
authors.lastname: Grant
authors.affiliation: Department of Neurosurgery, School of Medicine, Yale University, New Haven, CT, USA.
authors.forename: Kristopher T
authors.initials: KT
authors.lastname: Kahle
authors.affiliation: Department of Pediatrics and Hematology-Oncology, School of Medicine, Yale University, New Haven, CT, USA.
authors.forename: Asher M
authors.initials: AM
authors.lastname: Marks
authors.affiliation: Department of Neurosurgery, School of Medicine, Yale University, New Haven, CT, USA.
authors.forename: Michael L
authors.initials: ML
authors.lastname: DiLuna
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D004806:Ependymoma / Q000453:epidemiology* / Q000401:mortality* / Q000601:surgery; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D012189:Retrospective Studies; D018426:SEER Program / Q000706:statistics & numerical data*; D013120:Spinal Cord Neoplasms / Q000453:epidemiology* / Q000401:mortality* / Q000601:surgery; D055815:Young Adult
keywords: Ependymoma; Myxopapillary; Pediatric; SEER; Spinal cord tumor; Spine