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  • Child, Preschool  (90)
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  • 1
    Language: English
    In: World Neurosurgery, 2010, Vol.74(4), pp.532-537
    Description: A less favorable outcome is usually claimed for ETV in formerly shunted patients, and continuous bias exists on using endoscopy in cases with malfunctioning CSF shunts. A cohort of 60 patients with obstructive triventricular hydrocephalus (mean age 22 years, range 1–68) underwent an ETV instead of shunt revision. Fourteen patients had a history of multiple shunt-related surgeries (more than three times). Median follow-up lasted 2 years (range 1 month–8 years). Data on patients' preoperative condition and their history, including particularities of the surgery, were studied to define the impact of any given variable on the outcome. The Mann-Whitney test was used to assess differences among groups. Sixteen patients did not improve and needed permanent shunts anyway. The remaining 44 patients improved and became free of shunt (72%). No reliable correlation has been found regarding final outcome and data, characterizing patients' profile, for example, etiology of hydrocephalus, the history of intraventricular bleeding and/or CNS infection, age at onset and age at the first shunting, number of shunt surgeries, the origin of shunt malfunction, and complicated ventricular anatomy. There were no deaths, and overall cases with morbidity comprised 20% (12 cases); among them, serious complications with neurologic deficit were noted in three (5%) patients. Patients with obstructive hydrocephalus could benefit from ETV in case of their shunt malfunction and if carefully selected have about 70% probability to become shunt free. In formerly shunted patients, endoscopy has somewhat greater risk of serious complications; thus a wider experience is essential when offering them an ETV.
    Keywords: Cerebrospinal Fluid Shunts ; Endoscopic Third Ventriculostomy ; Obstructive Hydrocephalus ; Outcome
    ISSN: 1878-8750
    E-ISSN: 1878-8769
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  • 2
    Language: English
    In: Cancer, 15 March 2004, Vol.100(6), pp.1230-1237
    Description: BACKGROUND: Ependymomas account for 3-5% of all intracranial malignancies and occur most often in children and young adults. These neoplasms continue to generate considerable controversy with regard to their rational clinical management. It has been shown that the histologic classification of ependymoma is a significant predictor of clinical outcome in patients with ependymoma.METHODS: Ependymomas from 258 patients who underwent microsurgery at a single institution were evaluated histologically to elucidate the prognostic utility of a recently proposed grading scheme. Pathologic and clinical data then were compared using univariate and multivariate analyses.RESULTS: Increasing grade of ependymoma malignancy was found to be associated strongly and independently with worse clinical outcomes in terms of both event-free survival and overall survival. The effect of radiotherapy also was found to be related to histologic grade and was more beneficial for patients who had anaplastic ependymomas and had undergone complete tumor removal.CONCLUSIONS: The application of a uniform diagnostic criteria for grading ependymomas highlighted the key role of tumor histology in clinical outcome in a cohort of patients who were treated in the microsurgical era. The recently proposed grading scheme is likely to be practically useful, reproducible, and clinically applicable.
    Keywords: Ependymoma ; Histology ; Malignancy Grade ; Prognosis ; Radiotherapy
    ISSN: 0008-543X
    E-ISSN: 1097-0142
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  • 3
    Language: English
    In: International Journal of Cancer, 01 November 2011, Vol.129(9), pp.2297-2303
    Description: Pilocytic astrocytoma (PA) is emerging as a tumor entity with dysregulated Ras/Raf/MEK/ERK signaling. Common genetic lesions observed in PA, which are linked to aberrant ERK pathway activity, include either inactivation, or gain‐of‐function mutations. To investigate the mutation spectrum within the proto‐oncogene encoding the Ser/Thr‐kinase B‐Raf in more detail, we analyzed 64 primary tumor samples from children with PA including two patients with neurofibromatosis type 1 (NF1). The well‐known mutation was found in 6/64 (9.38%) of our samples. For the first time, we report concomitant presence of a somatic mutation in an NF1 patient indicating that more than one Ras/ERK pathway component can be affected in PA. Furthermore, 2/64 (3.13%) of our samples carried a 3‐bp insertion in resulting in the duplication of threonine 599. This conserved residue is located within the activation segment and, if phosphorylated in a Ras‐dependent manner, plays a key role in Raf activation. Here, we demonstrate that this mutant (B‐Raf) and another B‐Raf mutant, which carries two additional threonine residues at this position, display an kinase activity and cellular MEK/ERK activation potential comparable to those of B‐Raf. Notably, replacement of threonines by valine residues had similar effects on B‐Raf activity, suggesting that the distortion of the peptide backbone by additional amino acids rather than the insertion of additional, potential phosphorylation sites destabilizes the inactive conformation of the kinase domain. We also demonstrate that B‐Raf and B‐Raf, but not B‐Raf, provoke drastic morphological alterations in human astrocytes.
    Keywords: Pilocytic Astrocytoma ; Neurofibromatosis Type 1 ; B‐Raf ; Insertion Mutagenesis ; Mapk Pathway
    ISSN: 0020-7136
    E-ISSN: 1097-0215
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  • 4
    Language: English
    In: Zhurnal voprosy neirokhirurgii imeni N. N. Burdenko, 2016, Vol.80(4), pp.13-20
    Description: The study objective was to develop a rational approach for defining the extent of posterior decompression in children with Chiari 1 malformation. Posterior decompression was performed in 76 children with Chiari 1 malformation, under 18 years of age, in the period between 2001 and 2015. Fifty two (68%) children had syringomyelia. Extradural decompression (EDD) was performed in 14 (18%) cases, extra-arachnoid duraplasty (EAD) in 21 (28%) cases, intra-arachnoid dissection and duraplasty in 21 (28%) cases, and foramen of Magendie stenting and duraplasty in 20 (26%) cases. Complications occurred in 15 (20%) patients, with one of them being fatal (case fatality rate, 1.3%). The complication rate was higher after (1) intra-arachnoid dissection (p=0.0009) and stenting (p=0.02). Re-operation was required in 8 (11%) patients. The overall rate of complications and re-operations was lowest after EAD (10%). EAD is the method of choice for Chiari 1 malformation in children. EDD can be adopted as a primary option, but it requires selection of relevant patients. Intra-arachnoid dissection, with/without stenting, is not advisable as a primary intervention, but may be inevitable in the re-operation case.
    Keywords: Arnold-Chiari Malformation -- Surgery ; Decompression, Surgical -- Methods ; Syringomyelia -- Surgery
    ISSN: 0042-8817
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  • 5
    Language: English
    In: PLoS ONE, 01 January 2017, Vol.12(6), p.e0178351
    Description: Despite multimodal therapy, prognosis of pediatric intracranial ependymomas remains poor with a 5-year survival rate below 70% and frequent late deaths.This multicentric European study evaluated putative prognostic biomarkers. Tenascin-C (TNC) immunohistochemical expression and copy number status of 1q25 were retained for a pooled analysis of 5 independent cohorts. The prognostic value of TNC and 1q25 on the overall survival (OS) was assessed using a Cox model adjusted to age at diagnosis, tumor location, WHO grade, extent of resection, radiotherapy and stratified by cohort. Stratification on a predictor that did not satisfy the proportional hazards assumption was considered. Model performance was evaluated and an internal-external cross validation was performed.Among complete cases with 5-year median follow-up (n = 470; 131 deaths), TNC and 1q25 gain were significantly associated with age at diagnosis and posterior fossa tumor location. 1q25 status added independent prognostic value for death beyond the classical variables with a hazard ratio (HR) = 2.19 95%CI = [1.29; 3.76] (p = 0.004), while TNC prognostic relation was tumor location-dependent with HR = 2.19 95%CI = [1.29; 3.76] (p = 0.004) in posterior fossa and HR = 0.64 [0.28; 1.48] (p = 0.295) in supratentorial (interaction p value = 0.015). The derived prognostic score identified 3 different robust risk groups. The omission of upfront RT was not associated with OS for good and intermediate prognostic groups while the absence of upfront RT was negatively associated with OS in the poor risk group.Integrated TNC expression and 1q25 status are useful to better stratify patients and to eventually adapt treatment regimens in pediatric intracranial ependymoma.
    Keywords: Sciences (General)
    E-ISSN: 1932-6203
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  • 6
    Language: English
    In: Nature medicine, November 2018, Vol.24(11), pp.1752-1761
    Description: Brain tumors are the leading cause of cancer-related death in children. Genomic studies have provided insights into molecular subgroups and oncogenic drivers of pediatric brain tumors that may lead to novel therapeutic strategies. To evaluate new treatments, better preclinical models adequately reflecting the biological heterogeneity are needed. Through the Children's Oncology Group ACNS02B3 study, we have generated and comprehensively characterized 30 patient-derived orthotopic xenograft models and seven cell lines representing 14 molecular subgroups of pediatric brain tumors. Patient-derived orthotopic xenograft models were found to be representative of the human tumors they were derived from in terms of histology, immunohistochemistry, gene expression, DNA methylation, copy number, and mutational profiles. In vivo drug sensitivity of targeted therapeutics was associated with distinct molecular tumor subgroups and specific genetic alterations. These models and their molecular characterization provide an unprecedented resource for the cancer community to study key oncogenic drivers and to evaluate novel treatment strategies.
    Keywords: Biological Specimen Banks ; Immunohistochemistry ; Brain Neoplasms -- Pathology ; Xenograft Model Antitumor Assays -- Methods
    ISSN: 10788956
    E-ISSN: 1546-170X
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  • 7
    Language: English
    In: Acta Neuropathologica, 2011, Vol.122(6), pp.787-790
    Description: Byline: Adelheid Woehrer (1), Irene Slavc (2), Andreas Peyrl (2), Thomas Czech (3), Christian Dorfer (3), Daniela Prayer (4), Susanne Stary (5), Berthold Streubel (5), Marina Ryzhova (6), Andrey Korshunov (7), Stefan M. Pfister (8,9), Christine Haberler (1) Author Affiliation: (1) Institute of Neurology, Medical University of Vienna, Waehringer Guertel 18--20, 1097, Vienna, Austria (2) Department of Pediatrics, Medical University of Vienna, Vienna, Austria (3) Department of Neurosurgery, Medical University of Vienna, Vienna, Austria (4) Department of Radiology, Medical University of Vienna, Vienna, Austria (5) Department of Pathology, Medical University of Vienna, Vienna, Austria (6) NN Burdenko Neurosurgical Institute, Moscow, Russia (7) Clinical Cooperation Unit Neuropathology, German Cancer Research Center, Heidelberg, Germany (8) Division Molecular Genetics, German Cancer Research Center, Heidelberg, Germany (9) Department of Pediatric Oncology, Heidelberg University Hospital, Heidelberg, Germany Article History: Registration Date: 27/10/2011 Received Date: 07/10/2011 Accepted Date: 27/10/2011 Online Date: 06/11/2011
    Keywords: Cancer Research ; Genetic Research ; Tumors ; Medical Schools;
    ISSN: 0001-6322
    E-ISSN: 1432-0533
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  • 8
    Language: English
    In: Acta neuropathologica, September 2017, Vol.134(3), pp.507-516
    Description: Pediatric glioblastoma (pedGBM) is an extremely aggressive pediatric brain tumor, accounting for ~6% of all central nervous system neoplasms in children. Approximately half of pedGBM harbor recurrent somatic mutations in histone 3 variants or, infrequently, IDH1/2. The remaining subset of pedGBM is highly heterogeneous, and displays a variety of genomic and epigenetic features. In the current study, we aimed to further stratify an H3-/IDH-wild type (wt) pedGBM cohort assessed through genome-wide molecular profiling. As a result, we identified three molecular subtypes of these tumors, differing in their genomic and epigenetic signatures as well as in their clinical behavior. We designated these subtypes 'pedGBM_MYCN' (enriched for MYCN amplification), 'pedGBM_RTK1' (enriched for PDGFRA amplification) and 'pedGBM_RTK2' (enriched for EGFR amplification). These molecular subtypes were associated with significantly different outcomes, i.e. pedGBM_RTK2 tumors show a significantly longer survival time (median OS 44 months), pedGBM_MYCN display extremely poor outcomes (median OS 14 months), and pedGBM_RTK1 tumors harbor an intermediate prognosis. In addition, the various molecular subtypes of H3-/IDH-wt pedGBM were clearly distinguishable from their adult counterparts, underlining their biological distinctiveness. In conclusion, our study demonstrates significant molecular heterogeneity of H3-/IDH-wt pedGBM in terms of DNA methylation and cytogenetic alterations. The recognition of three molecular subtypes of H3-/IDH-wt pedGBM further revealed close correlations with biological parameters and clinical outcomes and may therefore, be predictive of response to standard treatment protocols, but could also be useful for stratification for novel, molecularly based therapies.
    Keywords: Brain Tumor ; Egfr ; Glioblastoma ; Mycn ; Methylation ; Pdgfra ; Pediatric ; Prognostic ; Rtk ; Subgroup ; Survival ; Mutation ; Brain Neoplasms -- Genetics ; Glioblastoma -- Genetics ; Histones -- Genetics ; Isocitrate Dehydrogenase -- Genetics
    ISSN: 00016322
    E-ISSN: 1432-0533
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  • 9
    In: Neuropathology and Applied Neurobiology, October 2013, Vol.39(6), pp.693-705
    Description: Byline: C. Colin, L. Padovani, C. Chappe, S. Mercurio, D. Scavarda, A. Loundou, F. Frassineti, N. Andre, C. Bouvier, A. Korshunov, G. Lena, D. Figarella-Branger Keywords: biological prognostic factors; clinicopathological prognostic factors; KIAA1549:BRAF fusion; pilocytic astrocytoma; pilomyxoid variant Background Pilocytic astrocytomas (PAs) are characterized by an excellent prognosis although several factors of adverse outcome have been reported. The mitogen-activated protein kinase pathway plays a major role in their tumorigenesis. Aim To report a series of 148 PAs in children to define clinicopathological and biological prognostic factors. Methods Clinical data were collected from patient files and mail inquiry. Pathological specimens were centrally reviewed. The three major KIAA1549:BRAF fusion subtypes were analysed by reverse transcription - polymerase chain reaction (RT-PCR) in a subset of 47 frozen cases and by fluorescence in situ hybridization on formalin-fixed paraffin-embedded tissue in 23 cases. Tumour location, age at surgery, extent of surgical removal, histological subtype and KIAA1549:BRAF fusion by RT-PCR were searched for prognostic significance. Results Pilomyxoid astrocytoma (PMA) and the hypothalamo-chiasmatic (H/C) location were associated with a worse prognosis [P 0.001 for overall survival (OS) and P=0.001 for progression-free survival (PFS)]. Patients who underwent complete surgical excision had a better OS (P=0.004) and a longer PFS (P 0.001) than the others. Age was also a strong prognostic factor for OS but not for PFS. Infants ( 1 year) and young children ( 3 years) had a much worse outcome than the others (P 0.001 and P=0.004 respectively). KIAA1549:BRAF fusion status was not predictive of outcome. Conclusion This study highlights the good prognostic factors of PAs but H/C PA remains a subgroup with dismal prognosis associated with young age, PMA variant and incomplete surgery. Search for KIAA1549:BRAF fusion in tumours with PA pattern is recommended even though the prognostic impact is still unclear. Article Note: These two authors have equally contributed to this work.
    Keywords: Biological Prognostic Factors ; Clinicopathological Prognostic Factors ; : Fusion ; Pilocytic Astrocytoma ; Pilomyxoid Variant
    ISSN: 0305-1846
    E-ISSN: 1365-2990
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  • 10
    Language: English
    In: Acta Neuropathologica, 2018, Vol.136(2), pp.273-291
    Description: Tumors with histological features of pilocytic astrocytoma (PA), but with increased mitotic activity and additional high-grade features (particularly microvascular proliferation and palisading necrosis) have often been designated anaplastic pilocytic astrocytomas. The status of these tumors as a separate entity has not yet been conclusively demonstrated and molecular features have only been partially characterized. We performed DNA methylation profiling of 102 histologically defined anaplastic pilocytic astrocytomas. T-distributed stochastic neighbor-embedding (t-SNE) and hierarchical clustering analysis of these 102 cases against 158 reference cases from 12 glioma reference classes revealed that a subset of 83 of these tumors share a common DNA methylation profile that is distinct from the reference classes. These 83 tumors were thus denominated DNA methylation class anaplastic astrocytoma with piloid features (MC AAP). The 19 remaining tumors were distributed amongst the reference classes, with additional testing confirming the molecular diagnosis in most cases. Median age of patients with MC AAP was 41.5 years. The most frequent localization was the posterior fossa (74%). Deletions of CDKN2A/B (66/83, 80%), MAPK pathway gene alterations (49/65, 75%, most frequently affecting NF1 , followed by BRAF and FGFR1 ) and mutations of ATRX or loss of ATRX expression (33/74, 45%) were the most common molecular alterations. All tumors were IDH1/2 wildtype. The MGMT promoter was methylated in 38/83 tumors (45%). Outcome analysis confirmed an unfavorable clinical course in comparison to PA, but better than IDH wildtype glioblastoma. In conclusion, we show that a subset of histologically defined anaplastic pilocytic astrocytomas forms a separate DNA methylation cluster, harbors recurrent alterations in MAPK pathway genes in combination with alterations of CDKN2A/B and ATRX , affects patients who are on average older than those diagnosed with PA and has an intermediate clinical outcome.
    Keywords: Anaplastic pilocytic astrocytoma ; Pilocytic astrocytoma with anaplasia ; Methylation profile based classification ; Panel sequencing ; ATRX ; BRAF ; NF1 ; FGFR1 ; MGMT ; CDKN2A/B ; Molecular characterization ; DNA copy number alterations
    ISSN: 0001-6322
    E-ISSN: 1432-0533
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