title: Preoperative chemotherapy in children with high-risk medulloblastomas: a feasibility study.
abstract: OBJECTIVE
  The authors set out to evaluate the feasibility and effectiveness of preoperative chemotherapy in treating high-risk medulloblastomas.
  
  
  METHODS
  Between 1997 and 2000, 21 children with high-risk medulloblastomas (M > or = 2 and/or T3b/T4 according to the Chang classification) were treated consecutively in a pilot study. The protocol began with treatment of the hydrocephalus and confirmation of the diagnosis. Tumor surgery was performed either after conventional chemotherapy (eight patients) or after subsequent high-dose chemotherapy (HDCT; 11 patients). Two children with early leptomeningeal progression died before surgery. Craniospinal irradiation was applied to children older than 5 years of age, whereas younger children received local irradiation only. Hydrocephalus was present in 17 children and was treated with ventriculocisternostomy in 13 and shunt insertion in four. A biopsy procedure was performed with a stereotactic frame in 10 children, an open surgery was performed in four, an endoscope was used during the ventriculocisternostomy in three, and the diagnosis was made based on cerebrospinal fluid cytological analysis in two. The response rate to the first two courses of chemotherapy was 71% for the tumor and 59% for the metastases. The pathological analysis of the residue after chemotherapy showed true medulloblastomas in seven cases, complete neuroglial maturation in three cases, and a mixture of both in nine cases. Three-year progression-free survival was 37% and was significantly better in children older than 5 years of age. There was one death related to the HDCT.
  
  
  CONCLUSIONS
  Preoperative chemotherapy is feasible and safe in children with high-risk medulloblastomas provided that the hydrocephalus can be treated at diagnosis. A larger study is warranted to ensure that the high response rate to adjuvant chemotherapy can lead to better surgical results and survival advantage.
authors.affiliation: Department de Cancérologie de l'Enfant et de l'Adolescent, Institut Gustave Roussy, Villejuif, France. grill@igr.fr
authors.forename: Jacques
authors.initials: J
authors.lastname: Grill
authors.forename: Arielle
authors.initials: A
authors.lastname: Lellouch-Tubiana
authors.forename: Selma
authors.initials: S
authors.lastname: Elouahdani
authors.forename: Alain
authors.initials: A
authors.lastname: Pierre-Kahn
authors.forename: Michel
authors.initials: M
authors.lastname: Zerah
authors.forename: Dominique
authors.initials: D
authors.lastname: Renier
authors.forename: Dominique
authors.initials: D
authors.lastname: Valteau-Couanet
authors.forename: Olivier
authors.initials: O
authors.lastname: Hartmann
authors.forename: Chantal
authors.initials: C
authors.lastname: Kalifa
authors.forename: Christian
authors.initials: C
authors.lastname: Sainte-Rose
mesh_terms: D002528:Cerebellar Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000150:complications / Q000601:surgery; D007223:Infant; D008297:Male; D008527:Medulloblastoma / Q000188:drug therapy* / Q000401:mortality / Q000532:radiotherapy / Q000601:surgery; D010865:Pilot Projects; D015996:Survival Rate; D014696:Ventriculostomy