title: Cervicomedullary tumors in children.
abstract: OBJECTIVE
  Cervicomedullary tumors (CMTs) represent a heterogeneous group of intrinsic neoplasms that are typically low grade and generally carry a good prognosis. This single-institution study was undertaken to document the outcomes and current treatment philosophy for these challenging neoplasms.
  
  
  METHODS
  The charts of all pediatric patients with CMTs who received treatment at St. Jude Children's Research Hospital between January 1988 and May 2013 were retrospectively reviewed. Demographic, surgical, clinical, radiological, pathological, and survival data were collected. Treatment-free survival and overall survival were estimated, and predictors of recurrence were analyzed.
  
  
  RESULTS
  Thirty-one children (16 boys, 15 girls) with at least 12 months of follow-up data were identified. The median age at diagnosis was 6 years (range 7 months-17 years) and the median follow-up was 4.3 years. Low-grade tumors (Grade I or II) were present in 26 (84%) patients. Thirty patients underwent either a biopsy alone or resection, with the majority of patients undergoing biopsy only (n = 12, 39%) or subtotal resection (n = 14, 45%). Only 4 patients were treated solely with resection; 21 patients received radiotherapy alone or in combination with other treatments. Recurrent tumor developed in 14 children (45%) and 4 died as a result of their malignancy. A high-grade pathological type was the only independent variable that predicted recurrence. The 5- and 10-year treatment-free survival estimates are 64.7% and 45.3%, respectively. The 5- and 10-year overall survival estimate is 86.7%.
  
  
  CONCLUSIONS
  Children with CMTs typically have low-grade neoplasms and consequently long-term survival, but high risk of recurrence. Therapy should be directed at achieving local tumor control while preserving and even restoring neurological function.
authors.affiliation: School of Medicine, Wake Forest University, Winston-Salem, North Carolina;
authors.forename: Joseph H
authors.initials: JH
authors.lastname: McAbee
authors.affiliation: University at Buffalo School of Medicine and Biomedical Sciences, Buffalo, New York;
authors.forename: Joseph
authors.initials: J
authors.lastname: Modica
authors.affiliation: George Washington University Milken Institute School of Public Health, Washington, DC; and.
authors.forename: Clinton J
authors.initials: CJ
authors.lastname: Thompson
authors.affiliation: Department of Oncology, St. Jude Children's Research Hospital;|Department of Pediatrics, University of Tennessee Health Science Center;
authors.forename: Alberto
authors.initials: A
authors.lastname: Broniscer
authors.affiliation: Department of Pathology, St. Jude Children's Research Hospital;
authors.forename: Brent
authors.initials: B
authors.lastname: Orr
authors.affiliation: Department of Neurosurgery, University of Tennessee Health Science Center;|Department of Radiology, University of Tennessee Health Science Center;|Le Bonheur Neuroscience Institute, Le Bonheur Children's Hospital;
authors.forename: Asim F
authors.initials: AF
authors.lastname: Choudhri
authors.affiliation: Department of Neurosurgery, University of Tennessee Health Science Center;|Le Bonheur Neuroscience Institute, Le Bonheur Children's Hospital;|Department of Surgery, St. Jude Children's Research Hospital; and.|Semmes-Murphey Neurologic & Spine Institute, Memphis, Tennessee.
authors.forename: Frederick A
authors.initials: FA
authors.lastname: Boop
authors.affiliation: Department of Neurosurgery, University of Tennessee Health Science Center;|Le Bonheur Neuroscience Institute, Le Bonheur Children's Hospital;|Department of Surgery, St. Jude Children's Research Hospital; and.|Semmes-Murphey Neurologic & Spine Institute, Memphis, Tennessee.
authors.forename: Paul
authors.initials: P
authors.lastname: Klimo
mesh_terms: D000293:Adolescent; D001706:Biopsy; D020295:Brain Stem Neoplasms / Q000188:drug therapy / Q000453:epidemiology* / Q000532:radiotherapy / Q000601:surgery; D002574:Cervical Vertebrae; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003663:Decision Trees; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D005910:Glioma / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D060787:Neoplasm Grading; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D019635:Neurosurgical Procedures; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D013120:Spinal Cord Neoplasms / Q000188:drug therapy / Q000453:epidemiology* / Q000532:radiotherapy / Q000601:surgery; D013123:Spinal Fusion; D016896:Treatment Outcome
keywords: CMT = cervicomedullary tumor; GBM = glioblastoma multiforme; GTR = gross-total resection; HGG NOS = high-grade glioma, not otherwise specified; NTR = near-total resection; OS = overall survival; PD = progressive disease; RT = radiotherapy; SD = stable disease; STR = subtotal resection; TFS = treatment-free survival; cervicomedullary tumor; chemotherapy; low-grade neoplasm; oncology; outcomes; radiation; resection