TY - JOUR
T1 - Childhood medulloblastoma
AU - Massimino, Maura
AU - Giangaspero, Felice
AU - Garrè, Maria Luisa
AU - Gandola, Lorenza
AU - Poggi, Geraldina
AU - Biassoni, Veronica
AU - Gatta, Gemma
AU - Rutkowski, Stefan
PY - 2011/7
Y1 - 2011/7
N2 - Among all the childhood central nervous system tumours, medulloblastoma and other neuroectodermal tumours account for 16-25% of cases. The causative factors of medulloblastoma/PNET have not been well established. It is more frequent in boys than in girl and in children than in adults. There was a significant improvement of survival for children diagnosed in 2000-2002 compared to those diagnosed in 1995-1999. The risk of dying was reduced by 30%. Patients are generally divided into risk-stratified schemes on the basis of age, the extent of residual disease, and dissemination. Sixty to 70% of patients older than 3 years are assigned to the average-risk group. High-risk patients include those in the disseminated category, and in North American trials those that have less than a gross or near-total resection, which is arbitrarily defined as 1.5cm
2 of post-operative residual disease. Current and currently planned clinical trials will:(1)evaluate the feasibility of reducing both the dose of craniospinal irradiation and the volume of the posterior fossa radiotherapy boost by the modest intensification of chemotherapy in standard-risk patients;(2)determine whether intensification of chemotherapy or irradiation can improve outcome in patients with high-risk disease;define molecular and biological markers that improve outcome prediction in patients with medulloblastoma and which can be incorporated for front-line stratification of newly defined risk subgroups.
AB - Among all the childhood central nervous system tumours, medulloblastoma and other neuroectodermal tumours account for 16-25% of cases. The causative factors of medulloblastoma/PNET have not been well established. It is more frequent in boys than in girl and in children than in adults. There was a significant improvement of survival for children diagnosed in 2000-2002 compared to those diagnosed in 1995-1999. The risk of dying was reduced by 30%. Patients are generally divided into risk-stratified schemes on the basis of age, the extent of residual disease, and dissemination. Sixty to 70% of patients older than 3 years are assigned to the average-risk group. High-risk patients include those in the disseminated category, and in North American trials those that have less than a gross or near-total resection, which is arbitrarily defined as 1.5cm
2 of post-operative residual disease. Current and currently planned clinical trials will:(1)evaluate the feasibility of reducing both the dose of craniospinal irradiation and the volume of the posterior fossa radiotherapy boost by the modest intensification of chemotherapy in standard-risk patients;(2)determine whether intensification of chemotherapy or irradiation can improve outcome in patients with high-risk disease;define molecular and biological markers that improve outcome prediction in patients with medulloblastoma and which can be incorporated for front-line stratification of newly defined risk subgroups.
KW - Late effects
KW - Medulloblastoma
KW - Pediatric brain tumors
KW - Prognosis
KW - Rehabilitation in brain tumors
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U2 - 10.1016/j.critrevonc.2010.07.010
DO - 10.1016/j.critrevonc.2010.07.010
M3 - Article
C2 - 21129995
AN - SCOPUS:79957933718
VL - 79
SP - 65
EP - 83
JO - Critical Reviews in Oncology/Hematology
JF - Critical Reviews in Oncology/Hematology
SN - 1040-8428
IS - 1
ER -