title: Myxopapillary ependymomas in children: imaging, treatment  and outcomes.
abstract: Myxopapillary ependymomas (MPEs) are rare spinal tumors in children. The natural history and clinical course of pediatric MPEs are largely unknown and the indication for adjuvant therapy remains to be clarified. We performed an IRB-approved, retrospective review of children with MPEs treated at the Dana-Farber/Boston Children's Cancer and Blood Disorder Center between 1982 and 2013. Eighteen children (age range 8-21 years, median age 14 years) met inclusion criteria. We reviewed the histopathology, magnetic resonance imaging, tumor location and stage, surgical management, adjuvant therapy, and clinical outcomes. The median follow-up duration was 9.4 years (range 1-30 years). Children most commonly presented with pain, scoliosis, and urinary symptoms. All primary tumors were located in the lower thoracic or lumbar spine. Nine children (50%) had leptomeningeal tumor seeding at presentation, most commonly located within the distal thecal sac. A gross-total resection was achieved in nine children (50%). Three children were treated with irradiation following initial surgery. No child received adjuvant chemotherapy at diagnosis. The 10-year event-free survival (EFS) was 26% ± 14.8. Children with disseminated disease trended towards inferior EFS compared to those with localized disease (10-year EFS 12.7% ± 12 vs. 57 ± 25%, p value 0.07). The 10-year overall survival was 100%. The efficacy of adjuvant irradiation could not be assessed due to the small sample size. Although children with MPEs frequently present with disseminated tumor and/or develop recurrent or progressive disease, their overall survival is excellent. Treatment should aim to minimize both tumor- and therapy-related morbidity.
authors.affiliation: Dana-Farber Cancer Institute and Boston Children's Hospital, Dana-Farber/Boston Children's Cancer and Blood Disorder Center, 450 Brookline Ave, Boston, 02215, USA.
authors.forename: Pratiti
authors.initials: P
authors.lastname: Bandopadhayay
authors.affiliation: Department of Radiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02215, USA.
authors.forename: V Michelle
authors.initials: VM
authors.lastname: Silvera
authors.affiliation: Division of Neuropathology, Department of Pathology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02215, USA.
authors.forename: Pedro D S C
authors.initials: PDSC
authors.lastname: Ciarlini
authors.affiliation: Dana-Farber Cancer Institute and Boston Children's Hospital, Dana-Farber/Boston Children's Cancer and Blood Disorder Center, 450 Brookline Ave, Boston, 02215, USA.
authors.forename: Hayley
authors.initials: H
authors.lastname: Malkin
authors.affiliation: Department of Neurosurgery, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02215, USA.
authors.forename: Wenya Linda
authors.initials: WL
authors.lastname: Bi
authors.affiliation: Dana-Farber Cancer Institute and Boston Children's Hospital, Dana-Farber/Boston Children's Cancer and Blood Disorder Center, 450 Brookline Ave, Boston, 02215, USA.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Bergthold
authors.affiliation: Dana-Farber Cancer Institute and Boston Children's Hospital, Dana-Farber/Boston Children's Cancer and Blood Disorder Center, 450 Brookline Ave, Boston, 02215, USA.
authors.forename: Ahmed M
authors.initials: AM
authors.lastname: Faisal
authors.affiliation: Dana-Farber Cancer Institute and Boston Children's Hospital, Dana-Farber/Boston Children's Cancer and Blood Disorder Center, 450 Brookline Ave, Boston, 02215, USA.|Department of Neurology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA, 02115, USA.
authors.forename: Nicole J
authors.initials: NJ
authors.lastname: Ullrich
authors.affiliation: Dana-Farber Cancer Institute and Boston Children's Hospital, Dana-Farber/Boston Children's Cancer and Blood Disorder Center, 450 Brookline Ave, Boston, 02215, USA.
authors.forename: Karen
authors.initials: K
authors.lastname: Marcus
authors.affiliation: Department of Neurosurgery, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02215, USA.
authors.forename: R Michael
authors.initials: RM
authors.lastname: Scott
authors.affiliation: Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Ave, Boston, MA, 02215, USA.
authors.forename: Rameen
authors.initials: R
authors.lastname: Beroukhim
authors.affiliation: Dana-Farber Cancer Institute and Boston Children's Hospital, Dana-Farber/Boston Children's Cancer and Blood Disorder Center, 450 Brookline Ave, Boston, 02215, USA.
authors.forename: Peter E
authors.initials: PE
authors.lastname: Manley
authors.affiliation: Dana-Farber Cancer Institute and Boston Children's Hospital, Dana-Farber/Boston Children's Cancer and Blood Disorder Center, 450 Brookline Ave, Boston, 02215, USA.
authors.forename: Susan N
authors.initials: SN
authors.lastname: Chi
authors.affiliation: Division of Neuropathology, Department of Pathology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02215, USA. Keith_Ligon@dfci.harvard.edu.|Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Ave, Boston, MA, 02215, USA. Keith_Ligon@dfci.harvard.edu.
authors.forename: Keith L
authors.initials: KL
authors.lastname: Ligon
authors.affiliation: Dana-Farber Cancer Institute and Boston Children's Hospital, Dana-Farber/Boston Children's Cancer and Blood Disorder Center, 450 Brookline Ave, Boston, 02215, USA. liliana.goumnerova@childrens.harvard.edu.|Department of Neurosurgery, Boston Children's Hospital, 300 Longwood Ave, Boston, MA, 02215, USA. liliana.goumnerova@childrens.harvard.edu.|Department of Neurology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA, 02115, USA. liliana.goumnerova@childrens.harvard.edu.
authors.forename: Liliana C
authors.initials: LC
authors.lastname: Goumnerova
authors.affiliation: Dana-Farber Cancer Institute and Boston Children's Hospital, Dana-Farber/Boston Children's Cancer and Blood Disorder Center, 450 Brookline Ave, Boston, 02215, USA. mark_kieran@dfci.harvard.edu.
authors.forename: Mark W
authors.initials: MW
authors.lastname: Kieran
mesh_terms: D000293:Adolescent; D016543:Central Nervous System Neoplasms / Q000473:pathology* / Q000628:therapy*; D002648:Child; D015331:Cohort Studies; D018572:Disease-Free Survival; D004806:Ependymoma / Q000473:pathology* / Q000628:therapy*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D016896:Treatment Outcome; D055815:Young Adult