Childhood medulloblastoma

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• 作者：Maura Massimino^a ; ^{START@istitutotumori.mi.it"" rel=""nofollow} ; Felice Giangaspero^b ; Maria Luisa Garr&#xe8 ; ^c ; Lorenza Gandola^a ; Geraldina Poggi^d ; Veronica Biassoni^a ; Gemma Gatta^a ; Stefan Rutkowski^e [Author vitae]
• 关键词：Medulloblastoma ; Pediatric brain tumors ; Prognosis ; Late effects ; Rehabilitation in brain tumors
• 刊名：Critical Reviews in Oncology/Hematology
• 出版年：2011
• 出版时间：July 2011
• 年：2011
• 卷：79
• 期：1
• 页码：65-83
• 全文大小：628 K

文摘

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 1.5 cm² 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.