Abstract

Medulloblastoma accounts for 15-20% of childhood nervous system tumours. The risk of dying was reduced by 30% in the last twenty years. Patients are divided in risk strata according to post-surgical disease, dissemination, histology and some molecular features such as WNT subgroup and MYC status. Sixty to 70% of patients older than 3 years are assigned to the average-risk group. High-risk patients include those with disseminated and/or residual disease, large cell and/or anaplastic histotypes, MYC genes amplification. 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 (RT) for those patients at low biologic risk, commonly identified as those having a medulloblastoma of the WNT subgroup;. (2) determine whether intensification of chemotherapy (CT) or irradiation can improve outcome in patients with high-risk disease;. (3) find target therapies allowing tailored therapies especially for relapsing patients and those with higher biological risk.

Original languageEnglish
JournalCritical Reviews in Oncology/Hematology
DOIs
Publication statusAccepted/In press - Oct 28 2015

Fingerprint

Medulloblastoma
Craniospinal Irradiation
Nervous System Neoplasms
Gene Amplification
Histology
Radiotherapy
Clinical Trials
Drug Therapy
Therapeutics

Keywords

  • Medulloblastoma
  • Molecular subgroups
  • Pediatric brain tumours
  • Prognosis
  • Rehabilitation in brain tumours
  • Target therapy

ASJC Scopus subject areas

  • Oncology
  • Hematology
  • Geriatrics and Gerontology

Cite this

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title = "Childhood medulloblastoma",
abstract = "Medulloblastoma accounts for 15-20{\%} of childhood nervous system tumours. The risk of dying was reduced by 30{\%} in the last twenty years. Patients are divided in risk strata according to post-surgical disease, dissemination, histology and some molecular features such as WNT subgroup and MYC status. Sixty to 70{\%} of patients older than 3 years are assigned to the average-risk group. High-risk patients include those with disseminated and/or residual disease, large cell and/or anaplastic histotypes, MYC genes amplification. 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 (RT) for those patients at low biologic risk, commonly identified as those having a medulloblastoma of the WNT subgroup;. (2) determine whether intensification of chemotherapy (CT) or irradiation can improve outcome in patients with high-risk disease;. (3) find target therapies allowing tailored therapies especially for relapsing patients and those with higher biological risk.",
keywords = "Medulloblastoma, Molecular subgroups, Pediatric brain tumours, Prognosis, Rehabilitation in brain tumours, Target therapy",
author = "Maura Massimino and Veronica Biassoni and Lorenza Gandola and Garr{\`e}, {Maria Luisa} and Gemma Gatta and Felice Giangaspero and Geraldina Poggi and Stefan Rutkowski",
year = "2015",
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AU - Massimino, Maura

AU - Biassoni, Veronica

AU - Gandola, Lorenza

AU - Garrè, Maria Luisa

AU - Gatta, Gemma

AU - Giangaspero, Felice

AU - Poggi, Geraldina

AU - Rutkowski, Stefan

PY - 2015/10/28

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N2 - Medulloblastoma accounts for 15-20% of childhood nervous system tumours. The risk of dying was reduced by 30% in the last twenty years. Patients are divided in risk strata according to post-surgical disease, dissemination, histology and some molecular features such as WNT subgroup and MYC status. Sixty to 70% of patients older than 3 years are assigned to the average-risk group. High-risk patients include those with disseminated and/or residual disease, large cell and/or anaplastic histotypes, MYC genes amplification. 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 (RT) for those patients at low biologic risk, commonly identified as those having a medulloblastoma of the WNT subgroup;. (2) determine whether intensification of chemotherapy (CT) or irradiation can improve outcome in patients with high-risk disease;. (3) find target therapies allowing tailored therapies especially for relapsing patients and those with higher biological risk.

AB - Medulloblastoma accounts for 15-20% of childhood nervous system tumours. The risk of dying was reduced by 30% in the last twenty years. Patients are divided in risk strata according to post-surgical disease, dissemination, histology and some molecular features such as WNT subgroup and MYC status. Sixty to 70% of patients older than 3 years are assigned to the average-risk group. High-risk patients include those with disseminated and/or residual disease, large cell and/or anaplastic histotypes, MYC genes amplification. 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 (RT) for those patients at low biologic risk, commonly identified as those having a medulloblastoma of the WNT subgroup;. (2) determine whether intensification of chemotherapy (CT) or irradiation can improve outcome in patients with high-risk disease;. (3) find target therapies allowing tailored therapies especially for relapsing patients and those with higher biological risk.

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