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1. Segmental chromosomal alterations have prognostic impact in neuroblastoma: a report from the INRG project.

Author

Schleiermacher, G, Mosseri, V, London, W, Maris, J, Brodeur, G, Attiyeh, E, Haber, M, Khan, J, Nakagawara, A, Speleman, F, Noguera, R, Tonini, G, Fischer, M, Ambros, I, Monclair, T, Matthay, Katherine, Ambros, P, Cohn, S, and Pearson, A

Subjects
Chromosome Aberrations, Chromosomes, Human, Pair 11, Chromosomes, Human, Pair 17, Humans, Infant, N-Myc Proto-Oncogene Protein, Neuroblastoma, Nuclear Proteins, Oncogene Proteins, Prognosis, Retrospective Studies, Survival Analysis
Abstract

BACKGROUND: In the INRG dataset, the hypothesis that any segmental chromosomal alteration might be of prognostic impact in neuroblastoma without MYCN amplification (MNA) was tested. METHODS: The presence of any segmental chromosomal alteration (chromosome 1p deletion, 11q deletion and/or chromosome 17q gain) defined a segmental genomic profile. Only tumours with a confirmed unaltered status for all three chromosome arms were considered as having no segmental chromosomal alterations. RESULTS: Among the 8800 patients in the INRG database, a genomic type could be attributed for 505 patients without MNA: 397 cases had a segmental genomic type, whereas 108 cases had an absence of any segmental alteration. A segmental genomic type was more frequent in patients >18 months and in stage 4 disease (P

Published
2012

2. Segmental chromosomal alterations have prognostic impact in neuroblastoma: A report from the INRG project

Author

Matthay, Katherine, Schleiermacher, G, Mosseri, V, London, WB, Maris, JM, Brodeur, GM, Attiyeh, E, Haber, M, Khan, J, Nakagawara, A, and Speleman, F

Abstract

Background: In the INRG dataset, the hypothesis that any segmental chromosomal alteration might be of prognostic impact in neuroblastoma without MYCN amplification (MNA) was tested. Methods: The presence of any segmental chromosomal alteration (chromosome

Published
2012

5. IL10RA modulates crizotinib sensitivity in NPM1-ALK+ anaplastic large cell lymphoma

Author

Prokoph, N, Probst, N, Lee, L, Monahan, J, Matthews, J, Liang, H, Bahnsen, K, Montes-Mojarro, I, Karaca-Atabay, E, Sharma, G, Malik, V, Larose, H, Forde, S, Ducray, S, Lobello, C, Wang, Q, Luan, S, Pospisilova, S, Gambacorti Passerini, C, Burke, G, Pervez, S, Attarbaschi, A, Janikova, A, Pacquement, H, Landman-Parker, J, Lambilliotte, A, Schleiermacher, G, Klapper, W, Jauch, R, Woessmann, W, Vassal, G, Kenner, L, Merkel, O, Mologni, L, Chiarle, R, Brugieres, L, Geoerger, B, Barbieri, I, Turner, S, Prokoph N., Probst N. A., Lee L. C., Monahan J. M., Matthews J. D., Liang H. -C., Bahnsen K., Montes-Mojarro I. A., Karaca-Atabay E., Sharma G. G., Malik V., Larose H., Forde S. D., Ducray S. P., Lobello C., Wang Q., Luan S. -L., Pospisilova S., Gambacorti Passerini C., Burke G. A. A., Pervez S., Attarbaschi A., Janikova A., Pacquement H., Landman-Parker J., Lambilliotte A., Schleiermacher G., Klapper W., Jauch R., Woessmann W., Vassal G., Kenner L., Merkel O., Mologni L., Chiarle R., Brugieres L., Geoerger B., Barbieri I., Turner S. D., Prokoph, N, Probst, N, Lee, L, Monahan, J, Matthews, J, Liang, H, Bahnsen, K, Montes-Mojarro, I, Karaca-Atabay, E, Sharma, G, Malik, V, Larose, H, Forde, S, Ducray, S, Lobello, C, Wang, Q, Luan, S, Pospisilova, S, Gambacorti Passerini, C, Burke, G, Pervez, S, Attarbaschi, A, Janikova, A, Pacquement, H, Landman-Parker, J, Lambilliotte, A, Schleiermacher, G, Klapper, W, Jauch, R, Woessmann, W, Vassal, G, Kenner, L, Merkel, O, Mologni, L, Chiarle, R, Brugieres, L, Geoerger, B, Barbieri, I, Turner, S, Prokoph N., Probst N. A., Lee L. C., Monahan J. M., Matthews J. D., Liang H. -C., Bahnsen K., Montes-Mojarro I. A., Karaca-Atabay E., Sharma G. G., Malik V., Larose H., Forde S. D., Ducray S. P., Lobello C., Wang Q., Luan S. -L., Pospisilova S., Gambacorti Passerini C., Burke G. A. A., Pervez S., Attarbaschi A., Janikova A., Pacquement H., Landman-Parker J., Lambilliotte A., Schleiermacher G., Klapper W., Jauch R., Woessmann W., Vassal G., Kenner L., Merkel O., Mologni L., Chiarle R., Brugieres L., Geoerger B., Barbieri I., and Turner S. D.

Abstract

Anaplastic large cell lymphoma (ALCL) is a T-cell malignancy predominantly driven by a hyperactive anaplastic lymphoma kinase (ALK) fusion protein. ALK inhibitors, such as crizotinib, provide alternatives to standard chemotherapy with reduced toxicity and side effects. Children with lymphomas driven by nucleophosmin 1 (NPM1)-ALK fusion proteins achieved an objective response rate to ALK inhibition therapy of 54% to 90% in clinical trials; however, a subset of patients progressed within the first 3 months of treatment. The mechanism for the development of ALK inhibitor resistance is unknown. Through genome-wide clustered regularly interspaced short palindromic repeats (CRISPR) activation and knockout screens in ALCL cell lines, combined with RNA sequencing data derived from ALK inhibitor-relapsed patient tumors, we show that resistance to ALK inhibition by crizotinib in ALCL can be driven by aberrant upregulation of interleukin 10 receptor subunit alpha (IL10RA). Elevated IL10RA expression rewires the STAT3 signaling pathway, bypassing otherwise critical phosphorylation by NPM1-ALK. IL-10RA expression does not correlate with response to standard chemotherapy in pediatric patients, suggesting that a combination of crizotinib and chemotherapy could prevent ALK inhibitor resistance-specific relapse.

Published
2020

9. Malformations, Genetic Abnormalities, and Wilms Tumor

Author

Dumoucel, S., Gauthier-Villars, M., Stoppa-Lyonnet, D., Parisot, P., Brisse, H., Philippe-Chomette, P., Sarnacki, S., Boccon-Gibod, L., Rossignol, S., Baumann, C., Aerts, I., Bourdeaut, F., Doz, F., Orbach, D., Pacquement, H., Michon, J., and Schleiermacher, G.

Published
2014
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10. The pitfalls and promise of liquid biopsies for diagnosing and treating solid tumors in children: a review

Author

Van Paemel, R., Vlug, R., De Preter, K., Van Roy, N., Speleman, F., Willems, L., Lammens, T., Laureys, G., Schleiermacher, G., Tytgat, G.A.M., Astrahantseff, K., Deubzer, H., and De Wilde, B.

Subjects
Cancer Research
Abstract

Cell-free DNA profiling using patient blood is emerging as a non-invasive complementary technique for cancer genomic characterization. Since these liquid biopsies will soon be integrated into clinical trial protocols for pediatric cancer treatment, clinicians should be informed about potential applications and advantages but also weaknesses and potential pitfalls. Small retrospective studies comparing genetic alterations detected in liquid biopsies with tumor biopsies for pediatric solid tumor types are encouraging. Molecular detection of tumor markers in cell-free DNA could be used for earlier therapy response monitoring and residual disease detection as well as enabling detection of pathognomonic and therapeutically relevant genomic alterations. CONCLUSION: Existing analyses of liquid biopsies from children with solid tumors increasingly suggest a potential relevance for molecular diagnostics, prognostic assessment, and therapeutic decision-making. Gaps remain in the types of tumors studied and value of detection methods applied. Here we review the current stand of liquid biopsy studies for pediatric solid tumors with a dedicated focus on cell-free DNA analysis. There is legitimate hope that integrating fully validated liquid biopsy-based innovations into the standard of care will advance patient monitoring and personalized treatment of children battling solid cancers.

Published
2020

11. Age Dependency of the Prognostic Impact of Tumor Genomics in Localized Resectable MYCN -Nonamplified Neuroblastomas. Report From the SIOPEN Biology Group on the LNESG Trials and a COG Validation Group

Author

Ambros IM, Tonini GP, Pötschger U, Gross N, Mosseri V, Beiske K, Berbegall AP, Bénard J, Bown N, Caron H, Combaret V, Couturier J, Defferrari R, Delattre O, Jeison M, Kogner P, Lunec J, Marques B, Martinsson T, Mazzocco K, Noguera R, Schleiermacher G, Valent A, Van Roy N, Villamon E, Janousek D, Pribill I, Glogova E, Attiyeh EF, Hogarty MD, Monclair TF, Holmes K, Valteau-Couanet D, Castel V, Tweddle DA, Park JR, Cohn S, Ladenstein R, Beck-Popovic M, De Bernardi B, Michon J, Pearson ADJ, and Ambros PF

Subjects
neoplasms
Abstract

For localized, resectable neuroblastoma without MYCN amplification, surgery only is recommended even if incomplete. However, it is not known whether the genomic background of these tumors may influence outcome.

Published
2020

13. Optimising Urinary Catecholamine Diagnostics for Neuroblastoma Patients

Author

Matser, Y., Verly, I., De Sain-Van der Velden, M., Wieczorek, A., Vicha, A., Ash, S., Pasqualini, C., Beck-Popovic, M., Schleiermacher, G., Canete, A., Owens, C., Ora, I., De Wilde, B., Cangemi, G., Garaventa, A., Grouzmann, E., Papadakis, V., Simon, T., Hero, B., Van Kuilenburg, A., Tytgat, G., Matser, Y., Verly, I., De Sain-Van der Velden, M., Wieczorek, A., Vicha, A., Ash, S., Pasqualini, C., Beck-Popovic, M., Schleiermacher, G., Canete, A., Owens, C., Ora, I., De Wilde, B., Cangemi, G., Garaventa, A., Grouzmann, E., Papadakis, V., Simon, T., Hero, B., Van Kuilenburg, A., and Tytgat, G.

Published
2020

16. Importance of Drug Formulation on the Pharmacokinetics of 13-CIS-Retinoic Acid (Isotretinoin) in Children with High-Risk Neuroblastoma

Author

MUNZER, C, Concordet, Didier, RUBIE, H, Gambart, M, DEFACHELLES, A.S, Schleiermacher, G, VEAL, G.L, CHU Toulouse [Toulouse], Innovations Thérapeutiques et Résistances (InTheRes), Institut National de la Recherche Agronomique (INRA)-Ecole Nationale Vétérinaire de Toulouse (ENVT), Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National Polytechnique (Toulouse) (Toulouse INP), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées, CLCC Oscar Lambret, Unite Oncol Pediat, Partenaires INRAE, Institut Curie [Paris], Newcastle University, and Société Internationale d’Oncologie Pédiatrique -SIOP.

Subjects
[SDV]Life Sciences [q-bio], ComputingMilieux_MISCELLANEOUS
Abstract

National audience

Published
2019

18. Age-dependency of the prognostic impact of tumor genomics in localized resectable MYCN non-amplified neuroblastomas Report from the SIOPEN Biology Group on the LNESG Trials

Author

Ambros, I.M., Tonini, G.P., Gross, N., Mosseri, V., Pötschger, U., Beiske, K., Berbegall, A.P., Bénard, J., Bown, N., Caron, H., Combaret, V., Couturier, J., Defferrari, R., Delattre, O., Jeison, M., Kogner, P., Lunec, J., Marques, B., Martinsson, T., Mazzocco, K., Noguera, R., Schleiermacher, G., Valent, A., Van Roy, N., Villamon, E., Janousek, D., Pribill, I., Glogova, E., Attiyeh, E.F., Hogarty, M.D., Monclair, T., Holmes, K., Valteau-Couanet, D., Pearson ADJ, A.D.J., Castel, V., Tweddle, D.A., Park, J.R., Cohn, S., Ladenstein, R., Beck-Popovic, M., De Bernardi, B., Michon, J., and Ambros, P.F.

Subjects
Neuroblastoma, Tumor Genomics, MYCN, Doenças Genéticas
Abstract

BACKGROUND: Biology based treatment reduction, i.e. surgery alone also in case of not totally resected tumors, was advised in neuroblastoma patients with localized resectable disease without MYCN amplification. However, whether the genomic background of these tumors may influence outcome was unknown and therefore scrutinized in a meta-analysis comprising two prospective therapy studies and a ‘validation’ cohort. PATIENTS AND METHODS: Diagnostic samples were derived from 406 INSS stages 1/2A/2B tumors from three cohorts: LNESGI/II and COG. Genomic data were analyzed in two age groups (cut-off: 18 months) and quality controlled by the SIOPEN Biology Group. RESULTS: In both patient age groups stage 2 tumors led to similarly reduced event-free survival (5y-EFS: 83+3% versus 80+4%), but overall survival was only decreased in patients >18m (5y-OS: 97+1% versus 87+4%; p=0.001). In the latter age subgroup, only tumors with SCA led to relapses, with 11q loss as the strongest marker (5y-EFS: 40+15% versus 89+5%; p18m but not

Published
2018

19. Data Descriptor: Meta-mining of copy number profiles of high-risk neuroblastoma tumors

Author

Depuydt, Pauline, Koster, J, Boeva, V, Hocking, TD, Speleman, Franki, Schleiermacher, G, and De Preter, Katleen

Subjects
AGE, MUTATIONS, Biology and Life Sciences
Abstract

Neuroblastoma, a pediatric tumor of the sympathetic nervous system, is predominantly driven by copy number aberrations, which predict survival outcome in global neuroblastoma cohorts and in low-risk cases. For high-risk patients there is still a need for better prognostic biomarkers. Via an international collaboration, we collected copy number profiles of 556 high-risk neuroblastomas generated on different array platforms. This manuscript describes the composition of the dataset, the methods used to process the data, including segmentation and aberration calling, and data validation. t-SNE analysis shows that samples cluster according to MYCN status, and shows a difference between array platforms. 97.3% of samples are characterized by the presence of segmental aberrations, in regions frequently affected in neuroblastoma. Focal aberrations affect genes known to be involved in neuroblastoma, such as ALK and L1N288. To conclude, we compiled a unique large copy number dataset of high-risk neuroblastoma tumors, available via R2 and a Shiny web application. The availability of patient survival data allows to further investigate the prognostic value of copy number aberrations.

Published
2018

20. Indications and results of diagnostic biopsy in pediatric renal tumors: A retrospective analysis of 317 patients with critical review of SIOP guidelines

Author

la Monneraye, Yvan, primary, Michon, J., additional, Pacquement, H., additional, Aerts, I., additional, Orbach, Daniel., additional, Doz, F., additional, Bourdeaut, F., additional, Sarnacki, S., additional, Philippe‐Chomette, P., additional, Audry, G., additional, Coulomb, A., additional, Fréneaux, P., additional, Klijanienko, J., additional, Berrebi, D., additional, Zucker, J.‐M., additional, Schleiermacher, G., additional, and Brisse, H.J., additional

Published
2019
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21. Monitoring of bone marrow minimal residual disease combined with genetic analysis identifies different risk groups in stage M neuroblastoma patients

Author

Riegler, S, additional, Ambros, IM, additional, Pötschger, U, additional, Mann, G, additional, Ziegler, A, additional, Modritz, D, additional, Crazzolara, R, additional, Ussowicz, M, additional, Benesch, M, additional, Ebetsberger-Dachs, G, additional, Schleiermacher, G, additional, Chan, GC, additional, Jones, N, additional, Yaniv, I, additional, Balwierz, W, additional, Holter, W, additional, Ladenstein, R, additional, and Ambros, PF, additional

Published
2017
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27. Segmental chromosomal alterations lead to a higher risk of relapse in infants with MYCN-non-amplified localised unresectable/disseminated neuroblastoma (a SIOPEN collaborative study). . 105:1940-1948,2011

Author

Schleiermacher, G, Michon, J, Ribeiro, A, Pierron, G, Mosseri, V, Rubie, H, Munzer, C, Bénard, J, Auger, N, Combaret, V, Janoueix Lerosey, I, Pearson, A, Tweddle, Da, Bown, N, Gerrard, M, Wheeler, K, Noguera, R, Villamon, E, Cañete, A, Castel, V, Marques, B, de Lacerda, A, Tonini, Gp, Mazzocco, K, Defferrari, R, de Bernardi, B, DI CATALDO, Andrea, van Roy, N, Brichard, B, Ladenstein, R, Ambros, I, Ambros, P, Beiske, K, Delattre, O, and Couturier, J.

Subjects
neuroblastoma, infants, genomic profile, segmental chromosome alterations, prognosis
Published
2011

29. Treatment and outcome of patients with relapsed clear cell sarcoma of the kidney: A combined SIOP and AIEOP study

Author

Gooskens, S.L.M. (Saskia), Furtwängler, R. (R.), Spreafico, F. (Filippo), Tinteren, H. (Harm) van, Kraker, J. (Jan) de, Vujanic, G.M. (Gordan), Leuschner, I. (Ivo), Coulomb-L'Herminé, A. (A.), Godzinski, J. (J.), Schleiermacher, G. (G.), Stoneham, S. (S.), Bergeron, C. (Christophe), Pritchard-Jones, K. (Kathy), Graf, N. (Norbert), Heuvel-Eibrink, M.M. (Marry) van den, Gooskens, S.L.M. (Saskia), Furtwängler, R. (R.), Spreafico, F. (Filippo), Tinteren, H. (Harm) van, Kraker, J. (Jan) de, Vujanic, G.M. (Gordan), Leuschner, I. (Ivo), Coulomb-L'Herminé, A. (A.), Godzinski, J. (J.), Schleiermacher, G. (G.), Stoneham, S. (S.), Bergeron, C. (Christophe), Pritchard-Jones, K. (Kathy), Graf, N. (Norbert), and Heuvel-Eibrink, M.M. (Marry) van den

Abstract

Background:Clear cell sarcoma of the kidney (CCSK) is an uncommon paediatric renal tumour. Relapses occur in about 15% of the patients. Since detailed clinical information on relapsed CCSK is scarce, the current study aims to describe outcome of patients with relapsed CCSK treated according to recent European protocols.Patients and methods:We analysed prospectively collected data of all CCSK patients who developed a relapse after complete remission at the end of primary treatment, entered onto SIOP and AIEOP trials between 1992 and 2012.Results:Thirty-seven of 237 CCSK patients (16%) treated according to SIOP and AIEOP protocols developed a relapse. Median time from initial diagnosis to relapse was 17 months (range, 5.5 months-6.6 years). Thirt-five out of thirty-seven relapses (95%) were metastatic; the most common sites of relapse were the brain (n=13), lungs (n=7) and bone (n=5). Relapse treatment consisted of chemotherapy (n=30), surgery (n=19) and/or radiotherapy (n=18), followed by high-dose chemotherapy and autologous bone marrow transplantation (ABMT) in 14 patients. Twenty-two out of thirty-seven patients (59%) achieved a second complete remission (CR); 15 of whom (68%) developed a second relapse. Five-year event-free survival (EFS) after relapse was 18% (95% CI: 4%-32%), and 5-year overall survival (OS) was 26% (95% CI: 10%-42%).Conclusions:In this largest series of relapsed CCSK patients ever described, overall outcome is poor. Most relapses are metastatic and brain relapses are more common than previously recognised. Intensive treatment aiming for local control, followed by high dose chemotherapy and ABMT, seems to be of benefit to enhance survival. Novel development of targeted therapy is urgently required.

Published
2014
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31. Influence of segmental chromosome abnormalities on survival in children over the age of 12 months with unresectable localised peripheral neuroblastic tumours without MYCN amplification

Author

Defferrari, R, primary, Mazzocco, K, additional, Ambros, I M, additional, Ambros, P F, additional, Bedwell, C, additional, Beiske, K, additional, Bénard, J, additional, Berbegall, A P, additional, Bown, N, additional, Combaret, V, additional, Couturier, J, additional, Erminio, G, additional, Gambini, C, additional, Garaventa, A, additional, Gross, N, additional, Haupt, R, additional, Kohler, J, additional, Jeison, M, additional, Lunec, J, additional, Marques, B, additional, Martinsson, T, additional, Noguera, R, additional, Parodi, S, additional, Schleiermacher, G, additional, Tweddle, D A, additional, Valent, A, additional, Van Roy, N, additional, Vicha, A, additional, Villamon, E, additional, and Tonini, G P, additional

Published
2014
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32. Treatment and outcome of patients with relapsed clear cell sarcoma of the kidney: a combined SIOP and AIEOP study

Author

Gooskens, S L, primary, Furtwängler, R, additional, Spreafico, F, additional, van Tinteren, H, additional, de Kraker, J, additional, Vujanic, G M, additional, Leuschner, I, additional, Coulomb-L'Herminé, A, additional, Godzinski, J, additional, Schleiermacher, G, additional, Stoneham, S, additional, Bergeron, C, additional, Pritchard-Jones, K, additional, Graf, N, additional, and van den Heuvel-Eibrink, M M, additional

Published
2014
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34. [Detection of micrometastases and circulating tumour cells using molecular biology technics in solid tumours]

Author

Schleiermacher G and olivier delattre

Subjects
Male, Reverse Transcriptase Polymerase Chain Reaction, Carcinoma, Prostatic Neoplasms, DNA, Neoplasm, Sarcoma, Ewing, Neoplastic Cells, Circulating, Polymerase Chain Reaction, Sensitivity and Specificity, Neuroblastoma, Biomarkers, Tumor, Humans, Neoplasm Metastasis, Melanoma
Abstract

The extension of a cancer is a major prognostic factor which determines the therapeutic strategy. The occurrence of metastatic relapses in patients with initially localized tumours, despite a good local control, gives evidence for the possibility of spreading of occult tumour cells. The recent improvements of immunohistochemistry and molecular biology methods enable to detect tumour cells in various sites such as lymph nodes, bone marrow and blood with a considerably increased sensitivity as compared to conventional approaches. The markers used to detect tumour cells by PCR or RT-PCR can be either "tissue-specific" or "tumour specific". The drawback of the first group of markers is linked to the observation that tissue-specificity is frequently a relative concept leading to a high rate of false positives. Tumour-specific markers include gene fusions observed in various sarcomas, point mutations and presence of viral genomes in tumour cells. They are available and can be easily monitored in only a limited set of cancers. This review focuses on the molecular biology approaches which are used to detect occult tumour cells and on their clinical applications. The large number of studies which have been published in that field show that such a detection can be performed in a variety of target sites. However, results of studies performed on larger series of patients together with a better standardization of technics are necessary before they can be used for individual staging of patients.

Published
2001

35. Segmental chromosomal alterations have prognostic impact in neuroblastoma: a report from the INRG project

Author

Schleiermacher, G., Mosseri, V., London, W. B., Maris, J. M., Brodeur, G. M., Attiyeh, E., Haber, M., Khan, J., Nakagawara, A., Speleman, F., Noguera, R., Tonini, G. P., Fischer, M., Ambros, I., Monclair, T., Matthay, K. K., Ambros, P., Cohn, S. L., Pearson, A. D. J., Schleiermacher, G., Mosseri, V., London, W. B., Maris, J. M., Brodeur, G. M., Attiyeh, E., Haber, M., Khan, J., Nakagawara, A., Speleman, F., Noguera, R., Tonini, G. P., Fischer, M., Ambros, I., Monclair, T., Matthay, K. K., Ambros, P., Cohn, S. L., and Pearson, A. D. J.

Abstract

BACKGROUND: In the INRG dataset, the hypothesis that any segmental chromosomal alteration might be of prognostic impact in neuroblastoma without MYCN amplification (MNA) was tested. METHODS: The presence of any segmental chromosomal alteration (chromosome 1p deletion, 11q deletion and/ or chromosome 17q gain) defined a segmental genomic profile. Only tumours with a confirmed unaltered status for all three chromosome arms were considered as having no segmental chromosomal alterations. RESULTS: Among the 8800 patients in the INRG database, a genomic type could be attributed for 505 patients without MNA: 397 cases had a segmental genomic type, whereas 108 cases had an absence of any segmental alteration. A segmental genomic type was more frequent in patients > 18 months and in stage 4 disease (P < 0.0001). In univariate analysis, 11q deletion, 17q gain and a segmental genomic type were associated with a poorer event-free survival (EFS) (P < 0.0001, P = 0.0002 and P < 0.0001, respectively). In multivariate analysis modelling EFS, the parameters age, stage and a segmental genomic type were retained in the model, whereas the individual genetic markers were not (P < 0.0001 and RR = 2.56; P = 0.0002 and RR = 1.8; P = 0.01 and RR = 1.7, respectively). CONCLUSION: A segmental genomic profile, rather than the single genetic markers, adds prognostic information to the clinical markers age and stage in neuroblastoma patients without MNA, underlining the importance of pangenomic studies. British Journal of Cancer (2012) 107, 1418-1422. doi:10.1038/bjc.2012.375 www.bjcancer.com Published online 13 September 2012 (c) 2012 Cancer Research UK

Published
2012

36. Segmental chromosomal alterations lead to a higher risk of relapse in infants with MYCN-non-amplified localised unresectable/disseminated neuroblastoma (a SIOPEN collaborative study).

Author

UCL - (SLuc) Service d'hématologie et d'oncologie pédiatrique, Schleiermacher, G, Michon, J, Ribeiro, A, Pierron, G, Mosseri, V, Rubie, H, Munzer, C, Bénard, J, Auger, N, Combaret, V, Janoueix-Lerosey, I, Pearson, A, Tweddle, D A, Bown, N, Gerrard, M, Wheeler, K, Noguera, R, Villamon, E, Cañete, A, Castel, V, Marques, B, de Lacerda, A, Tonini, G P, Mazzocco, K, Defferrari, R, de Bernardi, B, di Cataldo, A, van Roy, N, Brichard, Bénédicte, Ladenstein, R, Ambros, I, Ambros, P, Beiske, K, Delattre, O, Couturier, J, UCL - (SLuc) Service d'hématologie et d'oncologie pédiatrique, Schleiermacher, G, Michon, J, Ribeiro, A, Pierron, G, Mosseri, V, Rubie, H, Munzer, C, Bénard, J, Auger, N, Combaret, V, Janoueix-Lerosey, I, Pearson, A, Tweddle, D A, Bown, N, Gerrard, M, Wheeler, K, Noguera, R, Villamon, E, Cañete, A, Castel, V, Marques, B, de Lacerda, A, Tonini, G P, Mazzocco, K, Defferrari, R, de Bernardi, B, di Cataldo, A, van Roy, N, Brichard, Bénédicte, Ladenstein, R, Ambros, I, Ambros, P, Beiske, K, Delattre, O, and Couturier, J

Abstract

In infants with stage 4s MYCN-non-amplified NB, a SCA genomic profile identifies patients who will require upfront treatment even in the absence of other clinical indication for therapy, whereas in infants with localised unresectable NB, a genomic profile characterised by the absence of SCA identifies patients in whom treatment reduction might be possible. These findings will be implemented in a future international trial.

Published
2011

37. International consensus for neuroblastoma molecular diagnostics: report from the International Neuroblastoma Risk Group (INRG) Biology Committee

Author

Ambros, PF, Ambros, IM, Brodeur, GM, Haber, M, Khan, J, Nakagawara, A, Schleiermacher, G, Speleman, F, Spitz, R, London, WB, Cohn, SL, Pearson, ADJ, Maris, JM, Ambros, PF, Ambros, IM, Brodeur, GM, Haber, M, Khan, J, Nakagawara, A, Schleiermacher, G, Speleman, F, Spitz, R, London, WB, Cohn, SL, Pearson, ADJ, and Maris, JM

Abstract

Neuroblastoma serves as a paradigm for utilising tumour genomic data for determining patient prognosis and treatment allocation. However, before the establishment of the International Neuroblastoma Risk Group (INRG) Task Force in 2004, international consensus on markers, methodology, and data interpretation did not exist, compromising the reliability of decisive genetic markers and inhibiting translational research efforts. The objectives of the INRG Biology Committee were to identify highly prognostic genetic aberrations to be included in the new INRG risk classification schema and to develop precise definitions, decisive biomarkers, and technique standardisation. The review of the INRG database (n = 8800 patients) by the INRG Task Force finally enabled the identification of the most significant neuroblastoma biomarkers. In addition, the Biology Committee compared the standard operating procedures of different cooperative groups to arrive at international consensus for methodology, nomenclature, and future directions. Consensus was reached to include MYCN status, 11q23 allelic status, and ploidy in the INRG classification system on the basis of an evidence-based review of the INRG database. Standardised operating procedures for analysing these genetic factors were adopted, and criteria for proper nomenclature were developed. Neuroblastoma treatment planning is highly dependant on tumour cell genomic features, and it is likely that a comprehensive panel of DNA-based biomarkers will be used in future risk assignment algorithms applying genome-wide techniques. Consensus on methodology and interpretation is essential for uniform INRG classification and will greatly facilitate international and cooperative clinical and translational research studies.

Published
2009

38. Clear Cell Sarcomas of the Kidney registered on International Society of Pediatric Oncology (SIOP) 93-01 and SIOP 2001 protocols: A report of the SIOP Renal Tumour Study Group

Author

Furtwängler, R., primary, Gooskens, S.L., additional, van Tinteren, H., additional, de Kraker, J., additional, Schleiermacher, G., additional, Bergeron, C., additional, de Camargo, B., additional, Acha, T., additional, Godzinski, J., additional, Sandstedt, B., additional, Leuschner, I., additional, Vujanic, G.M., additional, Pieters, R., additional, Graf, N., additional, and van den Heuvel-Eibrink, M.M., additional

Published
2013
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39. Survie et toxicité tardive après radiothérapie pour un neuroblastome localisé. Dix ans d’expérience de la Société française de lutte contre les cancers de l’enfant (SFCE)

Author

Ducassou, A., primary, Gambart, M., additional, Munzer, C., additional, Carrie, C., additional, Claude, L., additional, Habrand, J., additional, Bolle, S., additional, Bernier, V., additional, Helfre, S., additional, Leseur, J., additional, Padovani, L., additional, Huchet, A., additional, Bergeron, C., additional, Valteau-Couanet, D., additional, Schleiermacher, G., additional, Coze, C., additional, Defachelles, A., additional, Plouvier, E., additional, Plantaz, D., additional, Perel, Y., additional, Devalck, C., additional, and Laprie, A., additional

Published
2013
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40. Malformations, genetic abnormalities, and wilms tumor

Author

Dumoucel, S., primary, Gauthier-Villars, M., additional, Stoppa-Lyonnet, D., additional, Parisot, P., additional, Brisse, H., additional, Philippe-Chomette, P., additional, Sarnacki, S., additional, Boccon-Gibod, L., additional, Rossignol, S., additional, Baumann, C., additional, Aerts, I., additional, Bourdeaut, F., additional, Doz, F., additional, Orbach, D., additional, Pacquement, H., additional, Michon, J., additional, and Schleiermacher, G., additional

Published
2013
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42. Segmental chromosomal alterations lead to a higher risk of relapse in infants with MYCN-non-amplified localised unresectable/disseminated neuroblastoma (a SIOPEN collaborative study)

Author

Schleiermacher, G, primary, Michon, J, additional, Ribeiro, A, additional, Pierron, G, additional, Mosseri, V, additional, Rubie, H, additional, Munzer, C, additional, Bénard, J, additional, Auger, N, additional, Combaret, V, additional, Janoueix-Lerosey, I, additional, Pearson, A, additional, Tweddle, D A, additional, Bown, N, additional, Gerrard, M, additional, Wheeler, K, additional, Noguera, R, additional, Villamon, E, additional, Cañete, A, additional, Castel, V, additional, Marques, B, additional, de Lacerda, A, additional, Tonini, G P, additional, Mazzocco, K, additional, Defferrari, R, additional, de Bernardi, B, additional, di Cataldo, A, additional, van Roy, N, additional, Brichard, B, additional, Ladenstein, R, additional, Ambros, I, additional, Ambros, P, additional, Beiske, K, additional, Delattre, O, additional, and Couturier, J, additional

Published
2011
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43. Mosaicism for oncogenic G12D KRAS mutation associated with epidermal nevus, polycystic kidneys and rhabdomyosarcoma

Author

Bourdeaut, F., primary, Herault, A., additional, Gentien, D., additional, Pierron, G., additional, Ballet, S., additional, Reynaud, S., additional, Paris, R., additional, Schleiermacher, G., additional, Baumann, C., additional, Philippe-Chomette, P., additional, Gauthier-Villars, M., additional, Peuchmaur, M., additional, Radvanyi, F., additional, and Delattre, O., additional

Published
2010
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46. Gene expression-based classification improves risk estimation of neuroblastoma patients

Author

Oberthuer, A, primary, Hero, B, additional, Juraeva, D, additional, Faldum, A, additional, Kahlert, Y, additional, Asgharzadeh, S, additional, Seeger, R, additional, Scaruffi, P, additional, Tonini, GP, additional, Janoueix-Lerosey, I, additional, Delattre, O, additional, Schleiermacher, G, additional, Vandesompele, J, additional, Vermeulen, J, additional, Speleman, F, additional, Noguera, R, additional, Piqueras, M, additional, Bénard, J, additional, Valent, A, additional, Avigad, S, additional, Yaniv, I, additional, Weber, A, additional, Christiansen, H, additional, Grundy, RG, additional, Schardt, K, additional, Schwab, M, additional, Eils, R, additional, Warnat, P, additional, Kaderali, L, additional, Simon, T, additional, DeCarolis, B, additional, Theissen, J, additional, Westermann, F, additional, Brors, B, additional, Berthold, F, additional, and Fischer, M, additional

Published
2010
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48. International consensus for neuroblastoma molecular diagnostics: report from the International Neuroblastoma Risk Group (INRG) Biology Committee

Author

Ambros, P F, primary, Ambros, I M, additional, Brodeur, G M, additional, Haber, M, additional, Khan, J, additional, Nakagawara, A, additional, Schleiermacher, G, additional, Speleman, F, additional, Spitz, R, additional, London, W B, additional, Cohn, S L, additional, Pearson, A D J, additional, and Maris, J M, additional

Published
2009
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49. International consensus for neuroblastoma molecular diagnostics: Report from the international neuroblastoma risk grouping (INRG) Biology committee

Author

Ambros, PF, primary, Ambros, IM, additional, Brodeur, GM, additional, Haber, M, additional, Khan, J, additional, Nakagawara, A, additional, Schleiermacher, G, additional, Speleman, F, additional, Spitz, R, additional, London, WB, additional, Cohn, SL, additional, Pearson, ADJ, additional, and Maris, JM, additional

Published
2009
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