title: Myxopapillary ependymoma in children: a study of 11 cases and a comparison with the adult experience.
abstract: BACKGROUND
  Myxopapillary ependymomas (MEPN) are uncommon tumors of the central nervous system, mainly occurring within the conus medullaris-filum terminale. MEPNs can also present in the skin and underlying soft tissues of the sacrococcygeal region. The incidence of extramedullary MEPNs in children is unclear.
  
  
  METHODS
  We retrospectively reviewed our institutional files for MEPN cases between the years 1990-2012. A total of 11 pediatric and 38 adult cases were identified and compared to determine differences between these two distinct age groups.
  
  
  RESULTS
  There were 40 (82%) tumors arising in the spine and nine (18%) in extramedullary sites. Pathologic examination revealed that extramedullary and spinal MEPNs were indistinguishable morphologically and immunophenotypically. Among the 11 children with MEPNs, seven had tumors presenting in extramedullary sites whereas only two adults (5%) had extramedullary tumors; this difference was highly significant (P < 0.0001). The lumbosacral portion of the spinal cord was the primary site for the remaining 40 MEPNs, four (10%) of which presented in children. One extramedullary MEPN (9%) recurred in a 45-year-old woman, and five (11%) of 40 spinal tumors recurred and/or metastasized; two of four children with spinal MEPNs developed recurrences and a manifestation to the aggressive nature of the latter tumor in the spinal cord of children.
  
  
  CONCLUSIONS
  Based upon our experience, MEPN in children is more likely to present in the extramedullary soft tissues of the sacrococcygeal region where its behavior is more indolent than those tumors arising in the spinal cord in children.
authors: Lauren V. Ackerman Laboratory of Surgical Pathology, St. Louis Children's Hospital, Washington University Medical Center, St. Louis, Missouri.
authors: Patrick J
authors: PJ
authors: Cimino
authors: Ashima
authors: A
authors: Agarwal
authors: Louis P
authors: LP
authors: Dehner
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D004806:Ependymoma / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012983:Soft Tissue Neoplasms / Q000473:pathology; D013120:Spinal Cord Neoplasms / Q000473:pathology
keywords: central nervous system; ependymoma; myxopapillary ependymoma; sacrococcygeal region; spinal cord