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  • 1
    In: Journal of Pediatric Hematology/Oncology, 2012, Vol.34(2), pp.e66-e68
    Description: We report on the first pediatric patient with a localized primary peripheral T-cell lymphoma, not otherwise specified, of the central nervous system (CNS). The solid lesion that was enhanced in magnetic resonance images of the left precentral region was totally resected. The histopathology revealed a peripheral T-cell lymphoma, not otherwise specified. Staging procedures showed that the lesion was confined to the CNS. Without any further therapy, the patient still remains in complete remission 6 years after diagnosis. Thus, we conclude that a peripheral T-cell lymphoma, not otherwise specified, of the CNS can occur in children. In the case presented here, complete resection sufficed.
    Keywords: Brain Neoplasms -- Pathology ; Lymphoma, T-Cell, Peripheral -- Pathology;
    ISSN: 1077-4114
    E-ISSN: 15363678
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  • 2
    Language: English
    In: International Journal of Radiation Oncology, Biology, Physics, 01 March 2012, Vol.82(3), pp.1135-1141
    Description: Therapy and tumor-related effects such as hypoperfusion, internal hydrocephalus, chemotherapy, and irradiation lead to significant motor and cognitive sequelae in pediatric posterior fossa tumor survivors. A distinct proportion of those factors related to the resulting late effects is hitherto poorly understood. This study aimed at separating the effects of neurotoxic factors on central nervous system metabolism by using H-1 MR spectroscopy to quantify cerebral metabolite concentrations in these patients in comparison to those in age-matched healthy peers. Fifteen patients with World Health Organization (WHO) I pilocytic astrocytoma (PA) treated by resection only, 24 patients with WHO IV medulloblastoma (MB), who additionally received chemotherapy and craniospinal irradiation, and 43 healthy peers were investigated using single-volume H-1 MR spectroscopy of parietal white matter and gray matter. Concentrations of -acetylaspartate (NAA) were significantly decreased in white matter ( 0.0001) and gray matter ( 0.0001) of MB patients and in gray matter ( 0.005) of PA patients, compared to healthy peers. Decreased creatine concentrations in parietal gray matter correlated significantly with older age at diagnosis in both patient groups (MB patients, 0.009, 0.52; PA patients, 0.006, 0.7). Longer time periods since diagnosis were associated with lower NAA levels in white matter of PA patients ( 0.008, 0.66). Differently decreased NAA concentrations were observed in both PA and MB groups of posterior fossa tumor patients. We conclude that this reflects a disturbance of the neurometabolic steady state of normal-appearing brain tissue due to the tumor itself and to the impact of surgery in both patient groups. Further incremental decreases of metabolite concentrations in MB patients may point to additional harm caused by irradiation and chemotherapy. The stronger decrease of NAA in MB patients may correspond to the additional damage of combined irradiation and chemotherapy on neuroaxonal cell viability and number.
    Keywords: Medulloblastoma ; Mr Proton Spectroscopy ; Neurometabolism ; Pilocytic Astrocytoma ; Sequelae ; Medicine
    ISSN: 0360-3016
    E-ISSN: 1879-355X
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  • 3
    In: Journal of Pediatric Hematology/Oncology, 2010, Vol.32(2), pp.156-159
    Description: Despite the growing knowledge on molecular risk factors of the most common malignant brain tumor in childhood, medulloblastoma, its biology remains only partially understood. A previous study investigating the entire mitochondrial genome of medulloblastoma revealed a number of somatic mutations in tumor and corresponding cerebrospinal fluid samples. In our present study we sought to corroborate these results on somatic and germ line mutations by comparing the complete mitochondrial genome sequences of medulloblastoma tissue in a further cohort of patients. Analysis of the entire mitochondrial genome by temporal temperature gel electrophoresis and direct sequencing revealed 6 somatic mutations in 6 of 15 medulloblastoma. All changes were insertions, deletions, or substitutions restricted to the np 303 to 315 poly-C tract of the D-loop region. Three were changes from heteroplasmy to homoplasmy. Two were changes from heteroplasmy to heteroplasmy and one mutation represented a change from homoplasmy to heteroplasmy. In addition, 25 distinct germ line variations were identified. These results are in support of our previous findings on frequency of somatic mitochondrial mutations in medulloblastoma. Somatic alterations were found only in the hypervariable D-loop region, supporting the idea that these control regions contain hot spots for both, germ line variations and somatic alterations of the mitochondrial genome.
    Keywords: Mutation ; Cerebellar Neoplasms -- Genetics ; DNA, Mitochondrial -- Genetics ; Medulloblastoma -- Genetics;
    ISSN: 1077-4114
    E-ISSN: 15363678
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  • 4
    Language: English
    In: International Journal of Radiation Oncology, Biology, Physics, 2010, Vol.76(3), pp.859-866
    Description: To elucidate morphologic correlates of brain dysfunction in pediatric survivors of posterior fossa tumors by using magnetic resonance diffusion tensor imaging (DTI) to examine neuroaxonal integrity in white matter. Seventeen medulloblastoma (MB) patients who had received surgery and adjuvant treatment, 13 pilocytic astrocytoma (PA) patients who had been treated only with surgery, and age-matched healthy control subjects underwent magnetic resonance imaging on a 3-Tesla system. High-resolution conventional T1- and T2-weighted magnetic resonance imaging and DTI data sets were obtained. Fractional anisotropy (FA) maps were analyzed using tract-based spatial statistics, a part of the Functional MRI of the Brain Software Library. Compared with control subjects, FA values of MB patients were significantly decreased in the cerebellar midline structures, in the frontal lobes, and in the callosal body. Fractional anisotropy values of the PA patients were not only decreased in cerebellar hemispheric structures as expected, but also in supratentorial parts of the brain, with a distribution similar to that in MB patients. However, the amount of significantly decreased FA was greater in MB than in PA patients, underscoring the aggravating neurotoxic effect of the adjuvant treatment. Neurotoxic mechanisms that are present in PA patients ( , internal hydrocephalus and damaged cerebellar structures affecting neuronal circuits) contribute significantly to the alteration of supratentorial white matter in pediatric posterior fossa tumor patients.
    Keywords: Diffusion Tensor Imaging ; Medulloblastoma ; Pilocytic Astrocytoma ; White Matter ; Sequelae ; Medicine
    ISSN: 0360-3016
    E-ISSN: 1879-355X
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  • 5
    Language: English
    In: Child's Nervous System, May, 2013, Vol.29(5), p.717(2)
    Description: Byline: Ulrich-Wilhelm Thomale (1), Pablo Hernaiz Driever (2) Author Affiliation: (1) Arbeitsbereich Padiatrische Neurochirurgie, Charite-Universitatsmedizin Berlin, Campus Virchow Klinik, Berlin, Germany (2) Klinik fur Padiatrie mit Schwerpunkt Onkologie und Hamatologie, Charite-Universitatsmedizin Berlin, Campus Virchow Klinik, Berlin, Germany Article History: Registration Date: 01/03/2013 Received Date: 25/02/2013 Accepted Date: 28/02/2013 Online Date: 16/03/2013
    ISSN: 0256-7040
    Source: Cengage Learning, Inc.
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  • 6
    Language: English
    In: Cancer, 01 February 2010, Vol.116(3), pp.705-712
    Description: BACKGROUND: The authors hypothesized that intensified chemotherapy in protocol HIT-GBM-C would increase survival of pediatric patients with high-grade glioma (HGG) and diffuse intrinsic pontine glioma (DIPG). METHODS: Pediatric patients with newly diagnosed HGG and DIPG were treated with standard fractionated radiation and simultaneous chemotherapy (cisplatin 20 mg/m2 X 5 days, etoposide 100 mg/m2 X 3 days, and vincristine, and 1 cycle of cisplatin + etoposide + ifosfamide 1.5 g/m X 5 days [PEI] during the last week of radiation). Subsequent maintenance chemotherapy included further cycles of PEI in Weeks 10, 14, 18, 22, 26, and 30, followed by oral valproic acid. RESULTS: Ninety-seven (pons, 37; nonpons, 60) patients (median age, 10 years; grade IV histology, 35) were treated. Resection was complete in 21 patients, partial in 29, biopsy only in 26, and not performed in 21. Overall survival rates were 91% (standard error of the mean [SE] ? 3%), 56%, and 19% at 6, 12, and 60 months after diagnosis, respectively. When compared with previous protocols, there was no significant benefit for patients with residual tumor, but the 5-year overall survival rate for patients with complete resection treated on HIT-GBM-C was 63% ? 12% SE, compared with 17% ? 10% SE for the historical control group (P = .003, log-rank test). CONCLUSIONS: HIT-GBM-C chemotherapy after complete tumor resection was superior to previous protocols. Cancer 2010.
    Keywords: Glioblastoma ; Diffuse Intrinsic Pontine Glioma ; Chemotherapy ; Resection ; Children
    ISSN: 0008-543X
    E-ISSN: 1097-0142
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  • 7
    Language: English
    In: Child's Nervous System, 2011, Vol.27(11), pp.1895-1902
    Description: Byline: Lindy Musial-Bright (1), Louisa Panteli (1), Pablo Hernaiz Driever (1) Keywords: Low-grade glioma; Childhood cancer survivors; Quality of life; Disability Abstract: Purpose The purpose of this study was to determine pediatric low-grade glioma survivors' quality of life and late morbidity including motor, sensory, and cognitive deficits. Methods We surveyed 49 survivors and their parents (KINDL questionnaire). Results Despite tumor and treatment-associated morbidity, survivors (25 boys and 24 girls, median age at diagnosis 7.8 years), but not their parents, rated their total quality of life higher than their peers. Although all survivors had some late morbidity, half of them were able to conduct their daily lives without restriction. Conclusion These results reflect survivors' effective coping mechanisms and underscore the difficulties of assessing quality of life in pediatric populations. Author Affiliation: (1) Department of Pediatric Oncology and Hematology, Charite-Universitatsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Germany Article History: Registration Date: 16/04/2011 Received Date: 24/01/2011 Accepted Date: 15/04/2011 Online Date: 03/05/2011
    Keywords: Low-grade glioma ; Childhood cancer survivors ; Quality of life ; Disability
    ISSN: 0256-7040
    E-ISSN: 1433-0350
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  • 8
    Language: English
    In: Cancer Research, 04/15/2011, Vol.71(8 Supplement), pp.3452-3452
    ISSN: 0008-5472
    E-ISSN: 1538-7445
    Source: CrossRef
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  • 9
    In: Journal of Pediatric Hematology/Oncology, 2009, Vol.31(10), pp.713-717
    Description: Serum levels of midkine (MK), a heparin-binding growth factor, are elevated in adult cancer patients. We analyzed sera of pediatric tumor patients in comparison to a large number of children and adolescents without malignant disease. MK was studied in sera of 152 noncancer patients and 29 embryonal tumor patients (14 nephroblastoma, 10 neuroblastoma, and 5 rhabdomyosarcoma) using an enzyme-linked immunosorbent assay. Noncancer patients underwent elective surgical procedures or suffered from an endocrinologic disease. They had no evidence of inflammation or injury. MK serum levels were significantly higher in tumor patients (median 0.621 ng/mL) than in noncancer patients. About 86% of tumor patients were identified using a cut-off value of 0.176 ng/mL. MK values did neither correlate with tumor size nor with stage or histology, but decreased in half of the nephroblastoma patients after chemotherapy and surgery. MK values were found to be elevated in only 2 out of 5 rhabdomyosarcoma patients. MK may serve as an additional marker for the detection of pediatric embryonal tumors, but its clinical relevance for the evaluation of response to therapy needs further study.
    Keywords: Neoplasms, Germ Cell and Embryonal -- Diagnosis ; Nerve Growth Factors -- Blood;
    ISSN: 1077-4114
    E-ISSN: 15363678
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  • 10
    Language: English
    In: Medical Microbiology and Immunology, 2011, Vol.200(1), pp.1-5
    Description: The question whether human cytomegalovirus may affect cancer diseases has been discussed (very controversially) for decades. There are convinced believers and strict opponents of the idea that HCMV might be able to play a role in the course of cancer diseases. In parallel, the number of published reports on the topic is growing. Recently published and presented (Ranganathan P, Clark P, Kuo JS, Salamat S, Kalejta RF. A Survey of Human Cytomegalovirus Genomic Loci Present in Glioblastoma Multiforme Tissue Samples. 35th Annual International Herpes Workshop, Salt Lake City, 2010) data on HCMV detection in glioblastoma tissues and colocalisation of HCMV proteins with cellular proteins known to be relevant for glioblastoma progression motivated us to recapitulate the current state of evidence.
    Keywords: Cytomegalovirus ; Cancer ; Oncomodulation ; Tumour virus ; Glioblastoma ; Neuroblastoma
    ISSN: 0300-8584
    E-ISSN: 1432-1831
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