title: Spinal glioblastoma multiforme of the conus medullaris with holocordal and intracranial spread in a child: a case report and review of the literature.
abstract: BACKGROUND
  Spinal glioblastoma multiforme (GBM) is a rare clinical entity. According to our review of the literature, only 15 cases of spinal GBM originating from the conus medullaris (CM) have been reported. Furthermore, there has been no case of spinal GBM originating from the CM with holocordal and intracranial involvements, which were already present at the time of initial diagnosis. Despite a variety of treatments, the previous studies have uniformly reported poor results of this lethal condition.
  
  
  OBJECTIVE
  The present report illustrates a 10-year-old girl with spinal GBM with rare involvement pattern, that is, the tumor originating from the CM with the holocordal and intracranial involvements, undergoing a novel chemotherapy regimen.
  
  
  METHODS
  A case report and review of literature.
  
  
  METHODS
  Magnetic resonance (MR) imaging with gadolinium enhancement clearly revealed holocordal and intracranial lesions, which were otherwise unidentifiable by plane MR imaging. Open biopsy was performed. After histologic diagnosis, novel chemotherapy regimen, that is, simultaneous high-dose chemotherapy (cyclophosphamide, cisplatin, vincristine, and etoposide) combined with autologous peripheral blood stem cell transplantation (auto-PBSCT), intrathecal injections of both methotrexate and dexamethasone, and radiotherapy, which respected the tolerance threshold of the spinal cord, were performed.
  
  
  RESULTS
  Novel chemotherapy regimen achieved marked tumor regression until the 12th month of treatment. The patient became ambulatory with T-shaped canes and has returned to the school life. Unfortunately, the patient died because of the relapse of the tumor 14 months after the initial diagnosis; however, this strategy has achieved longer survival than previously reported mean survival (12 months).
  
  
  CONCLUSIONS
  The authors advocate enhanced MR imaging of the whole central nervous system for the potential spreading of this disease. This is the first report of simultaneous high-dose chemotherapy combined with auto-PBSCT, intrathecal injections of antineoplastic agents, and radiotherapy for the treatment of spinal GBM, which achieved marked tumor regression. We believe that accumulated experiences in the treatment of this lethal condition might contribute well to improve its therapeutic outcome.
authors.affiliation: Department of Orthopaedic Surgery, Shiga University of Medical Science, Tsukinowa-cho, Seta, Otsu, Shiga 520-2192, Japan. kanchi@belle.shiga-med.ac.jp
authors.forename: Kanji
authors.initials: K
authors.lastname: Mori
authors.forename: Shinji
authors.initials: S
authors.lastname: Imai
authors.forename: Junji
authors.initials: J
authors.lastname: Shimizu
authors.forename: Takashi
authors.initials: T
authors.lastname: Taga
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Ishida
authors.forename: Yoshitaka
authors.initials: Y
authors.lastname: Matsusue
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000556:secondary* / Q000628:therapy; D002420:Cauda Equina / Q000473:pathology; D002648:Child; D003131:Combined Modality Therapy; D003287:Contrast Media; D017809:Fatal Outcome; D005260:Female; D005682:Gadolinium; D005909:Glioblastoma / Q000556:secondary* / Q000628:therapy; D006801:Humans; D007089:Image Enhancement / Q000379:methods; D008279:Magnetic Resonance Imaging; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D036102:Peripheral Blood Stem Cell Transplantation; D011878:Radiotherapy; D013120:Spinal Cord Neoplasms / Q000473:pathology* / Q000628:therapy
chemical_list: D003287:Contrast Media; D005682:Gadolinium