p53 expression predicts dismal outcome for medulloblastoma patients with metastatic disease.

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p53 expression predicts dismal outcome for medulloblastoma patients with metastatic disease. / Gessi, Marco; von Bueren, André; Rutkowski, Stefan; Rutkowski, Stefan; Pietsch, Torsten.

in: J NEURO-ONCOL, Jahrgang 106, Nr. 1, 1, 2012, S. 135-141.

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@article{c0fb32efbf454835bdc979b99ad2fee2,
title = "p53 expression predicts dismal outcome for medulloblastoma patients with metastatic disease.",
abstract = "Medulloblastoma (MB) is the most common malignant primary brain tumour in childhood. Metastatic disease (M+) at diagnosis is the most important negative prognostic clinical marker and, despite craniospinal irradiation and intensive chemotherapy, it remains one of the leading causes of treatment failure. To date, few clinical and biological data have been evaluated to obtain an additional prognostic profile for these high-risk patients. In this study, 169 patients with metastatic MB registered in the multicentre HIT2000 trial of the German Society of Pediatric Oncology and Haematology (GPOH) have been investigated to determine the importance of p53 protein expression in predicting survival. At a median follow-up of 4.1 years, 159 patients with p53-negative tumours had significantly better four-year event-free survival (EFS) and progression-free survival (PFS) (56 ± 11, 59 ± 4%) than 10 patients with p53-positive tumours (40 ± 16, 40 ± 16%; P = 0.018 for EFS, P = 0.007 for PFS, respectively). Furthermore, four-year overall survival (OS) of children with p53-negative tumours was higher than for children with p53-positive tumours (72 ± 4 vs. 35 ± 18%, P = 0.05). Three of the p53-positive MBs harbored a point mutation in the TP53 gene. p53 protein assessment by immunohistochemistry may be a useful tool for sub-stratification of metastatic high-risk MB patients.",
keywords = "Humans, Male, Female, Adolescent, Young Adult, Child, Immunohistochemistry, Predictive Value of Tests, Survival Analysis, Disease-Free Survival, Child, Preschool, Infant, Mutation, Tumor Markers, Biological, Point Mutation, Cerebellar Neoplasms/diagnosis/*genetics, DNA, Neoplasm/biosynthesis/genetics, Gene Expression Regulation, Neoplastic/*genetics, Genes, myc/genetics, Genes, p53/*genetics, Medulloblastoma/diagnosis/*genetics, Neoplasm Metastasis/*genetics/pathology, Polymorphism, Single-Stranded Conformational, beta Catenin/genetics, Humans, Male, Female, Adolescent, Young Adult, Child, Immunohistochemistry, Predictive Value of Tests, Survival Analysis, Disease-Free Survival, Child, Preschool, Infant, Mutation, Tumor Markers, Biological, Point Mutation, Cerebellar Neoplasms/diagnosis/*genetics, DNA, Neoplasm/biosynthesis/genetics, Gene Expression Regulation, Neoplastic/*genetics, Genes, myc/genetics, Genes, p53/*genetics, Medulloblastoma/diagnosis/*genetics, Neoplasm Metastasis/*genetics/pathology, Polymorphism, Single-Stranded Conformational, beta Catenin/genetics",
author = "Marco Gessi and {von Bueren}, Andr{\'e} and Stefan Rutkowski and Stefan Rutkowski and Torsten Pietsch",
year = "2012",
language = "English",
volume = "106",
pages = "135--141",
journal = "J NEURO-ONCOL",
issn = "0167-594X",
publisher = "Kluwer Academic Publishers",
number = "1",

}

RIS

TY - JOUR

T1 - p53 expression predicts dismal outcome for medulloblastoma patients with metastatic disease.

AU - Gessi, Marco

AU - von Bueren, André

AU - Rutkowski, Stefan

AU - Rutkowski, Stefan

AU - Pietsch, Torsten

PY - 2012

Y1 - 2012

N2 - Medulloblastoma (MB) is the most common malignant primary brain tumour in childhood. Metastatic disease (M+) at diagnosis is the most important negative prognostic clinical marker and, despite craniospinal irradiation and intensive chemotherapy, it remains one of the leading causes of treatment failure. To date, few clinical and biological data have been evaluated to obtain an additional prognostic profile for these high-risk patients. In this study, 169 patients with metastatic MB registered in the multicentre HIT2000 trial of the German Society of Pediatric Oncology and Haematology (GPOH) have been investigated to determine the importance of p53 protein expression in predicting survival. At a median follow-up of 4.1 years, 159 patients with p53-negative tumours had significantly better four-year event-free survival (EFS) and progression-free survival (PFS) (56 ± 11, 59 ± 4%) than 10 patients with p53-positive tumours (40 ± 16, 40 ± 16%; P = 0.018 for EFS, P = 0.007 for PFS, respectively). Furthermore, four-year overall survival (OS) of children with p53-negative tumours was higher than for children with p53-positive tumours (72 ± 4 vs. 35 ± 18%, P = 0.05). Three of the p53-positive MBs harbored a point mutation in the TP53 gene. p53 protein assessment by immunohistochemistry may be a useful tool for sub-stratification of metastatic high-risk MB patients.

AB - Medulloblastoma (MB) is the most common malignant primary brain tumour in childhood. Metastatic disease (M+) at diagnosis is the most important negative prognostic clinical marker and, despite craniospinal irradiation and intensive chemotherapy, it remains one of the leading causes of treatment failure. To date, few clinical and biological data have been evaluated to obtain an additional prognostic profile for these high-risk patients. In this study, 169 patients with metastatic MB registered in the multicentre HIT2000 trial of the German Society of Pediatric Oncology and Haematology (GPOH) have been investigated to determine the importance of p53 protein expression in predicting survival. At a median follow-up of 4.1 years, 159 patients with p53-negative tumours had significantly better four-year event-free survival (EFS) and progression-free survival (PFS) (56 ± 11, 59 ± 4%) than 10 patients with p53-positive tumours (40 ± 16, 40 ± 16%; P = 0.018 for EFS, P = 0.007 for PFS, respectively). Furthermore, four-year overall survival (OS) of children with p53-negative tumours was higher than for children with p53-positive tumours (72 ± 4 vs. 35 ± 18%, P = 0.05). Three of the p53-positive MBs harbored a point mutation in the TP53 gene. p53 protein assessment by immunohistochemistry may be a useful tool for sub-stratification of metastatic high-risk MB patients.

KW - Humans

KW - Male

KW - Female

KW - Adolescent

KW - Young Adult

KW - Child

KW - Immunohistochemistry

KW - Predictive Value of Tests

KW - Survival Analysis

KW - Disease-Free Survival

KW - Child, Preschool

KW - Infant

KW - Mutation

KW - Tumor Markers, Biological

KW - Point Mutation

KW - Cerebellar Neoplasms/diagnosis/genetics

KW - DNA, Neoplasm/biosynthesis/genetics

KW - Gene Expression Regulation, Neoplastic/genetics

KW - Genes, myc/genetics

KW - Genes, p53/genetics

KW - Medulloblastoma/diagnosis/genetics

KW - Neoplasm Metastasis/genetics/pathology

KW - Polymorphism, Single-Stranded Conformational

KW - beta Catenin/genetics

KW - Humans

KW - Male

KW - Female

KW - Adolescent

KW - Young Adult

KW - Child

KW - Immunohistochemistry

KW - Predictive Value of Tests

KW - Survival Analysis

KW - Disease-Free Survival

KW - Child, Preschool

KW - Infant

KW - Mutation

KW - Tumor Markers, Biological

KW - Point Mutation

KW - Cerebellar Neoplasms/diagnosis/genetics

KW - DNA, Neoplasm/biosynthesis/genetics

KW - Gene Expression Regulation, Neoplastic/genetics

KW - Genes, myc/genetics

KW - Genes, p53/genetics

KW - Medulloblastoma/diagnosis/genetics

KW - Neoplasm Metastasis/genetics/pathology

KW - Polymorphism, Single-Stranded Conformational

KW - beta Catenin/genetics

M3 - SCORING: Journal article

VL - 106

SP - 135

EP - 141

JO - J NEURO-ONCOL

JF - J NEURO-ONCOL

SN - 0167-594X

IS - 1

M1 - 1

ER -