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.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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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 -